Thrombopenic purpura in children. Thrombocytopenic purpura is a dangerous blood disorder. Traditional methods of treatment

Thrombocytopenic purpura is a rare skin disease that belongs to the group of hemorrhagic diathesis. With this pathology, a person produces an insufficient number of certain blood cells, most often their deficiency is caused by immune disorders.

Usually, the disease affects children under the age of 10 years, in adults, the disease occurs 2 times less often. The main symptom of this pathology is the appearance a large number hemorrhages on the skin and mucous membranes.

Characteristics of the disease

Werlhof disease - what is it? The disease refers to hematological diseases, in which there is a decrease in the number of platelets. Patients who have been diagnosed with it tend to bleed and may also develop hemorrhagic syndrome.

In thrombocytopenic purpura, the platelet count is significantly reduced and is usually about 150 × 109 L, while the number of megakaryocytes in the bone marrow is close to normal. Today, this pathology is considered quite common and most often manifests itself in the fair sex.

Thrombocytopenia is often diagnosed in patients who suffer from accelerated cell destruction... The method of treatment is selected depending on how much the disease is developed and how much the platelet count has dropped.

Symptoms

In children and adults, the disease manifests itself in a similar way. Since she does not have pronounced signs, for staging accurate diagnosis it is necessary to do a general analysis of blood and urine, as well as undergo some examinations.

The main symptoms of thrombocytopenia are:

the appearance of bruises on the skin different sizes; bruising can occur not only as a result of blows, but also completely suddenly, without outside influence;
profuse nosebleeds;
punctate hemorrhages in the upper layers of the epidermis and periodic - in the mucous membranes of the eyes;
frequent dizziness;
increased bleeding of the gums when brushing teeth;
periodic appearance of blood clots in the urine and feces, vomiting;
an increase in the size of the spleen (observed in the autoimmune form of the disease);
kidney dysfunction, which develops if the disease is in advanced form.

If a child suffers from thrombocytopenia, there is increased likelihood enlargement of lymph nodes and the appearance of pain during palpation.

Classification and forms

In international medicine, thrombocytopenic purpura is divided into several different forms depending on what became the primary source of the pathology and determined the clinical picture of the disease.

Classification according to the course and duration of the disease:

Sharp. Most often diagnosed in children under 8 years of age. The exacerbation of the disease disappears when the platelet count rises to normal (this usually takes about 6 months). With this form, relapses are extremely rare, more often it occurs full recovery.
Chronic. Usually diagnosed in adult patients, the duration of the disease is more than 6 months.
Recurrent. Depending on the patient's condition, the platelet level will periodically normalize, then decrease again.

Also, this disease is classified according to the mechanism of occurrence. After conducting examinations, doctors can give patients a specific diagnosis:

Idiopathic form. It is she who is known as Werlhof's disease, arises for no apparent reason (it is almost impossible to establish them).
Isoimmune. It develops if the patient receives frequent blood transfusions.
Transimmune. It occurs in the fetus while it is in the womb. The disease is usually transmitted through antiplatelet antibodies. In most cases, the value of the indicators drops significantly only in the first month of life, after which it normalizes.
Heteroimmune. The antigenic structure of platelets changes due to the fact that they are exposed to negative impact alien agents. This form of the disease can develop due to certain viral diseases or under the influence of medications. In the event that the disease does not go away for more than 6 months, it is transferred to an autoimmune form.
Autoimmune. It arises as a result of various internal pathologies, for example, after lupus erythematosus.

TO a separate category includes thrombotic purpura. This type of disease is always severe and has an unfavorable prognosis for the patient. Most often, the disease develops in patients aged 30 to 40 years.

The peculiarity of thrombotic purpura is that damaged platelets begin to stick together and clog the vessels, which can provoke ischemia internal organs... The disease primarily affects the lungs, kidneys, heart and brain.

Acute form the disease quickly begins to progress, with it, the patient begins to suffer from profuse bleeding, fever, tremors, seizures and visual disturbances.

All these symptoms together often lead to coma.... According to statistics, this form of the disease is fatal in 60% of cases.

Stages of pathology

The severity of symptoms may differ depending on how much the platelet count has dropped. Based on this indicator, the stages of the disease are distinguished:

Lightweight. With this degree of the disease, platelets decrease slightly. Due to the lack of pronounced signs, the patient will not even guess about the presence of the disease. The only thing that may indicate a pathology is periodic bleeding.
Average. At this stage, a person has rashes all over the body, and can also occur on the mucous membranes.
Heavy. Rashes cover most body, gastrointestinal bleeding appears.

The main causes of the disease

Despite numerous clinical researches and a comparison of various factors, leading physicians still cannot say for sure what the pathogenesis of purpura is. In about 45% of cases, doctors cannot identify what triggers the development of the disease.

Today, most experts believe that thrombocytopenia occurs against the background of factors such as:

wrong job circulatory system;
the formation of benign and malignant neoplasms in the bone marrow;
genetic predisposition;
constant stress and emotional stress;
severe frostbite;
capillary prosthetics operations;
ultraviolet irradiation;
physical damage;
radiation sickness;
long-term intake certain medical supplies, especially the negative effect of treatment antibacterial agents and estrogen-based drugs.

The risk group includes patients who have suffered diseases such as:

whooping cough;
chicken pox;
measles rubella;
Infectious mononucleosis;
flu.

The likelihood of developing the disease increases in people suffering from spleen dysfunction and those who have inhibited serotonin synthesis.

Diagnostics

In order to choose the optimal treatment method, the physician must take into account all the symptoms and factors in the aggregate. For determining clinical picture conduct an external therapeutic examination, collecting anamnesis, make an examination of the patient. Looking for:

a general blood test to determine the concentration of platelets;
biochemical analysis urine;
myelonogram.

If the patient suffers from increased frequency of nose or heavy uterine bleeding, this is also considered a sign of the progression of the disease. The final diagnosis is made by the hematologist after examining the analyzes. The doctor will assess how much the blood counts have changed.

For purple, deviations are inherent:

A sharp decrease in the level of platelets.
An increase in the size of these cells and a change in their structure.
Decrease in hemoglobin.
Increased bleeding time.
The presence of antiplatelet antibodies in the patient's blood.

Differential diagnosis is prescribed if there is a suspicion of secondary purpura.

What treatment is used

If a person's immune system malfunctions and he has thrombocytopenic purpura, it is necessary to start treatment as soon as possible in order to prevent the transition of the disease to the next stage.

V medical practice sometimes there are cases when the disease proceeds in isolation... At the same time, there is mild bleeding, a moderate level of platelet reduction. In such situations, treatment is usually not given. The main task of the patient at this stage is to prevent the progression of the disease.

If the patient was diagnosed with moderate or difficult stage thrombocytopenia, prescribe medication of certain pharmaceutical groups.

Also, treatment can be carried out using methods such as:

... A procedure that helps clear excess antibodies from the blood.
Transfusion of open erythrocytes and plasma (this method is especially effective for large blood loss).
If the disease is difficult to treat, the patient may be ordered to remove the spleen.

Each of these methods has its own advantages and weaknesses... The method of therapy should be selected by the attending physician, depending on the individual characteristics of the organism.

It is important to note that the treatment of the disease at the initial stage is carried out exclusively in a hospital. For a speedy recovery, the patient will need to comply with bed rest and the clinical recommendations of the doctor.

Drug therapy

Drug treatment is considered the mainstay of thrombocytopenic purpura. Typically, the patient is prescribed the following drugs:

Corticosteroids. Discharged with profuse hemorrhages, delay autoimmune processes.
Immunosuppressants Medicines of this pharmaceutical group suppress autoimmune reactions.
Means that accelerate blood circulation.
Antiplatelet agents. Drugs that reduce the likelihood of blood clots forming.

If conservative method treatment will be ineffective, the only way to save the patient's life is surgery.

Nutritional support

With thrombocytopenia, no strict diet is prescribed. The most important role is played by proper nutrition, a person's diet must be ideally balanced. The consumed meals should be easily absorbed by the body, saturate it with various vitamins and microelements.

Most doctors advise their patients to eat as many foods as possible that help thin the blood and prevent blood clots from forming.

It is necessary to exclude spicy and hot dishes from the diet, due to which the gastric mucosa can be damaged.

To prevent thrombocytopenia from progressing, and the condition to improve, it is necessary to consume:

buckwheat and oatmeal;
corn;
beef liver;
sprouted wheat;
vegetables and fruits;
nuts.

Traditional methods of treatment

Many patients still prefer to use home treatments because they find them safer. Doctors warn that it will not be possible to get rid of purpura solely with the help of folk recipes; they can only be used as an adjunct to drug therapy.

The most effective are:

A decoction of viburnum bark. 2 tablespoons of dry bark pour 300 ml of boiling water and put to simmer on a small fire. After the broth boils, turn it off and let it brew for 2 hours. The tool is taken three times a day, 50 ml.
Sesame powder. A small handful of seeds should be crushed and the resulting powder should be taken in a tablespoon 3 times a day (it should be washed down with plenty of water).
Barberry tincture. 20 g of chopped barberry leaves should be poured with 250 ml of water and placed in a dark place for 2 weeks, shaking occasionally. After this time, the medicine will be ready, it is taken three times a day, 5 ml.

Possible complications

Whatever the reasons for thrombocytopenia, this disease is considered very dangerous. In the absence of timely treatment, there is a possibility that a cerebral hemorrhage will occur. With thrombocytopenia, a stroke can occur completely suddenly, without any prerequisites.

If the patient suffers from heavy bleeding, after a few months he may be diagnosed with anemia or iron deficiency anemia. Children under 10 years of age with this ailment often develop stomatitis, gingivitis.

Today, thrombocytopenia responds well to treatment, with only 20% of patients diagnosed with death. But in any case, at the first signs of the disease, you must immediately consult a doctor and undergo a comprehensive diagnosis.

Diseases skin type affect children and adults at the same frequency. One of these ailments is thrombocytopenic purpura. Photo will be discussed in the article. The disease is caused by a complex nature and a special course. Let's consider how this process takes place in children, how often it forms in adults, and what treatment methods are considered the most effective for eliminating the disease.

Thrombocytopenic purpura what is it

Thrombocytopenic purpura, the photo of which is presented below, is one of the varieties of the hemorrhagic phenomenon - diathesis. The phenomenon of a deficiency of red blood platelets - platelets, is characteristic of the phenomenon, which is often caused by the influence of immune mechanisms. As signs of the disease are arbitrary effusions of blood into the skin, while they can be multiple or single. Also, the disease is accompanied by an increase in uterine, nasal and other blood flows. If there is a suspicion of progression this ailment, the task is to assess clinical data and other diagnostic information - ELISA, analyzes, smears and punctures.

This benign pathological is accompanied by the fact that there is a deficiency of platelets in the blood, and there is a tendency to increased bleeding and development hemorrhagic symptom... In the presence of an ailment in the peripheral blood system, a downward change in the level of blood plates is observed. Among all hemorrhagic types purpura diathesis is found most often, mainly in children of the preschool age factor. In adults and adolescents pathological syndrome is less common and mainly affects women.

The course of the disease can be divided into acute and chronic forms. In the first case, the disease occurs in childhood and lasts six months, while platelets are normalized, and there are no relapses. In the second situation, the duration of the disease exceeds a period of 6 months and progresses in adults. At the same time, the recurrent form has a cyclical course, with repetitive functions of individual episodes.

Causes of the disease

In 45% of situations, the disease develops on a spontaneous basis. In 40% of cases, the development of this pathology is preceded by numerous viral and bacterial infectious phenomena that were experienced a couple of weeks before the onset. Usually, several diseases can be distinguished among these factors.

chickenpox;
measles, rubella;
malaria;
whooping cough;
endocarditis.

Often, the manifestation of the disease occurs in the framework of active or passive immunization. A few more factors provoking the formation of the disease - reception medications, X-ray exposure, exposure chemical substances, surgical interventions, insolation.

Thrombocytopenic Purpura Symptoms

The onset of any disease and manifestation is especially acute. The sudden onset of hemorrhage aggravates the situation and creates a characteristic of the disease appearance cover. Against the background of primary symptoms, additional signs- nosebleeds, deterioration of the general condition, pallor of the skin, a sharp increase in bodily temperature regime... If this is an acute process, complete recovery without relapse usually occurs after 1 month. Against the background of the manifestation of the disease, many children are faced with a noticeable increase in lymph nodes. By easy reason course in about 50% of children, the disease cannot be diagnosed.

If we are talking about a chronic form of the disease, sharp start not provided. First symptoms appear long before acute pronounced signs... Bleeding occurs in the area of the skin and looks like punctate hemorrhages. They aggravate the overall picture with an exacerbation of the disease. In the first place among the formations is purpura of the skin - or an adult. Hemorrhagic manifestations usually appear abruptly, mainly at night.

In rare situations, “bloody tears” can be identified as a symptom, which is caused by profuse bleeding from the eye area. Usually this phenomenon is observed together with skin lesions. Blood goes persistently and abundantly, against the background of this phenomenon, anemia may occur. Gastrointestinal bleeding are less common, but in girls the process may be accompanied by uterine pathologies and secretions. Rarely, with purpura, hematomas and prolonged blood flow may occur. Children suffering from the disease usually have no complaints, except that a couple of signs can be distinguished.

General state of lethargy and apathy;
increased fatigue;
normal body temperature;
normal size of the spleen and liver;
sharp and severe irritability.

Chronic forms of the disease usually occur in children after 7 years of age. The course is undulating, when remissions and exacerbations alternate.

Thrombocytopenic purpura in children photo

Thrombocytopenic purpura, a photo of which is presented in the article, has several forms and manifestations. The main signs of its presence in young children are hemorrhages and effusions in the area of mucous membranes and under the skin. The color of hemorrhages varies from red to green, and the size that the body has, starts from small to significant blisters and spots.

The manifestation is characterized by the absence of symmetry and the logic of the sequence of education. In children, education does not hurt or bother. When is the stage of remission clinical type, the disease disappears and disappears completely.

Cutaneous hemorrhagic syndrome has several specific and characteristic manifestations.

The severity of the hemorrhagic symptom does not correspond to the traumatic effect. Formations can appear abruptly and unexpectedly.
During the course of childhood illness, polymorphism is observed, in which the variety and size indicators of rashes are different and multifaceted.
Cutaneous hemorrhage has no favorite localization sites. For example, it can appear on the body, face, and other parts of the body.

The most dangerous manifestation of childhood illness is the appearance of hemorrhage in the sclera, which can give rise to the most severe complication- cerebral hemorrhage. The outcome of this course depends on the form and location of the pathology, as well as on the therapeutic measures taken. Speaking about the prevalence of the disease, it can be noted that its frequency ranges from 10 to 125 cases per million population per year.

Thrombocytopenic purpura in children treatment

Before prescribing effective treatment methods, a study of the disease is carried out - a detailed diagnosis. It is possible that a decrease in the number of platelets will be found in the blood and longest time bleeding. There is not always a correspondence between the duration this phenomenon and the degree of thrombocytopenia. The altered properties of blood, changes in hormones in it, as well as other substances act as secondary indicators of the fact of the presence of a disease.

Differential diagnosis

The disease must be differentiated from a number of other manifestations that are similar in their signs and symptomatic indicators.

Acute leukemia - in in this case manifested metaplasia in the area bone marrow.
SLE - for a detailed diagnosis, it is necessary to take into account information from an immunological study.
Thrombocytopathy involves a change in the content and level of platelets in the opposite direction.

Treatment activities for children

Thrombocytopenic purpura, a photo of which is presented in the article, assumes no treatment if the forms of the disease are not severe. With moderate severity, therapeutic interventions are indicated if there is an increased risk of bleeding. Such phenomena are predominantly characteristic of ulcerative diseases, hypertension, duodenal ulcer. If the platelet count in the analysis reaches a value<20-30×10^9/л, то терапия назначается в стационарных условиях. Если больной человек страдает от хронической формы проявления заболевания, для которой характерны обильные течения крови и попадание жидкости в жизненно важные элементы, проводится спленэктомия. Ребенок во время геморрагического криза должен проводить время в постели.

If the therapy is pathogenetic, glucocorticosteroids are prescribed at 2 mg / kg of body weight per day by the oral route, immunosuppressants are used (cyclophosphamide, vincristine, if other methods are ineffective). All funds are specifically prescribed by the doctor.
To eliminate the symptoms and features of the manifestation of the disease, human Ig is used, while the child is prescribed a dosage of 1 g per kg of body weight for two days.
Danazol, which has an androgenic effect, is often used, as well as drugs of the interferon group - ROFERON-A, REAFERON, ANTI-A.
The remedies of the symptomatic group are aminocaproic acid, ethamsylate.

Forecast factors

In most situations, the prognosis is favorable. In children, full recovery occurs in 90% of cases, in adults - in 75%. At acute stage during the course of the process, noticeable complications in the form of a hemorrhagic stroke may occur, while there is a high risk of death. When carrying out treatment, it is necessary that the sick person is constantly under the supervision of a hematologist, while it is important to exclude from the diet treatment means that affect the properties of platelet aggregation. These drugs include caffeine, aspirin, barbiturate.

Thrombocytopenic purpura in adults photo

Along with other symptoms, it also takes place. The clinical picture is similar. Spots and formations on the skin do not bother a person. The only caveat is that thrombocytopenic purpura (see the photo in the article) among the adult population is 2-3 times more likely to affect the fair sex.

Thrombocytopenic purpura in adults treatment

The medical complex in children and adults has many similarities, and all therapeutic methods are determined by how difficult it is, and how it should last. Treatment consists of conservative therapy, which involves taking medications, as well as the use of surgical methods. Regardless of the manifestation of the disease, bed rest is an important part of recovery.

Drug treatment

Previously, remedies for treating an ailment in children were described, in principle, all of them are suitable for adults, but in order to simplify the situation and improve the condition, it is recommended to include in the diet hormonal drugs, contributing to a decrease in the vascular permeability index and an increase in blood clotting. If there are multiple, as well as other symptoms, the use of several groups of funds is effective.

PREDISOLON applied at 2 mg per kg of body weight per day, subsequently the dosage is reduced. Usually, this therapy gives a tremendous effect, in patients after hormone withdrawal, a relapse occurs.
If the measures taken in adults have not yielded results, use immunosuppressants... This imuran, 2-3 mg per kg of body weight per day. The duration of therapy is up to 5 months. Also vincristine 1-2 mg once a week.

The effect of such treatment can be detected already after a few weeks, over time the dosage is reduced. Patients should also use a vitamin complex, especially groups P, C. Such measures will make it possible to make noticeable improvements in the properties of platelets. In addition, doctors prescribe calcium salts, aminocaproic acids, ATP, magnesium sulfate. Collections of natural folk remedies (nettle, rose hips, pepper, St. John's wort) are effective.

If conservative methods of therapy are not particularly effective, it comes to resorting to removing the spleen by surgical methods. Often, cytostatic agents are prescribed to reduce the number of harmful cells. But these drugs are used exclusively in rare cases ( IMURAN, VINKRISTIN, CYCLOPHOSPHANE).

Diet for thrombocytopenic purpura

The main requirement, which assumes the possibility of quickly getting rid of the disease, is adherence to a special diet. The patient's diet should be moderately nutritious and complete. Food is served chilled, it should be liquid and contain small portions. which was presented in the article can quickly disappear if you use the right foods.

Food to lower platelets

There is no specific medical menu, but the main rule is to get complete food with proteins and essential vitamins. The benefit lies in the use of several product groups.

Buckwheat porridge;
peanuts and other nuts;
fruits and juices from them;
any vegetables;
corn;
beef liver;
oat flakes;
wheat and products from it.

What to exclude from the diet

spicy and salty foods;
high fat foods;
food with impurities and flavorings;
hot spices and sauces;
restaurant food and fast food products.

Do you know the disease thrombocytopenic purpura? Did the photo help with the definition of symptoms? Leave your feedback for everyone on the forum!

2. After vaccination (BCG).

3. Hypothermia or excessive sun exposure.

4. Injuries and surgical interventions.

5. As a result of the use of certain drugs:

Under the influence of the above factors, there is a direct decrease in the number of platelets or the formation of antibodies to platelets. Antigens in the form of viruses, vaccine components, drugs are attached to platelets, and the body begins to produce antibodies. Eventually, antibodies attach to antigens on top of platelets, forming an antigen-antibody complex. The body seeks to destroy these complexes, which is what happens in the spleen. Thus, the duration of platelet existence is reduced to 7-10 days. A decrease in the number of platelets in the blood leads to damage to the walls of blood vessels, which is manifested by bleeding, a change in vascular contractility and a violation of the formation of a blood clot.

Symptoms

Stages of thrombocytopenic purpura

2. Clinical remission- there are no visible clinical manifestations, but changes in the blood persist.

3. Clinical and hematological remission- restoration of laboratory blood parameters against the background of the absence of visible manifestations of the disease.

Diagnostics

a general blood test with counting the number of platelets;
determination of antiplatelet antibodies in the blood and Coombs' test;
bone marrow puncture;
determination of APTT, prothrombin time, fibrinogen level;
biochemical blood test (creatinine, urea, ALT, AST);
Wasserman reaction, determination of antibodies in the blood to the Epstein-Barr virus, parvovirus.

The diagnosis of "thrombocytopenic purpura" is made in the absence of clinical data indicating the presence of oncological diseases of the blood and systemic diseases. Thrombocytopenia is most often not accompanied by a decrease in erythrocytes and leukocytes.

Thrombocytopenic purpura in children

Treatment

Drug therapy

Oral hormones are prescribed as follows:

The total dose is prednisolone at a dose of 1-2 mg / kg per day for 21 days, then the dose is gradually reduced until it is completely canceled. A second course is possible in a month.
In high doses, prednisolone at a dose of 4-8 mg / kg per day is taken for a week, or methylprednisolone at a dose of mg / kg per day, with a quick subsequent withdrawal of the drug, a second course is carried out after 1 week.
"Pulse therapy" with hydrocortisone - 0.5 mg / kg per day, taken 4 days after 28 days (the course is 6 cycles).

Methylprednisolone is administered intravenously g / kg per day, from 3 to 7 days in severe disease.

Normal human immunoglobulin for intravenous administration;
Intraglobin F;
Octagam;
Sandoglobulin;
Venoglobulin, etc.

In the acute form, immunoglobulins are prescribed at a dose of 1 g / kg per day for 1 or 2 days. In the chronic form, a single administration of the drug is subsequently prescribed to maintain the required platelet level.

It is indicated for chronic purpura in case of ineffectiveness of glucocorticoid treatment. 2x106 IU of interferon-alpha is injected under the skin or into the muscle for a month, 3 times a week, every other day.

Vincristine and Vinblastine are administered within a month, once a week.

It must be remembered about the possibility of tissue necrosis with the introduction of pink periwinkle alkaloids subcutaneously. Perhaps a violation of nerve conduction, the appearance of foci of alopecia (alopecia).

Apply the drug by mouth, at a dose of mg / kg per day. It is necessary to divide the dose into 3 doses, the course is 3 months.

Shows a toxic effect on the liver, possibly weight gain during treatment, the appearance of excessive hair growth (hirsutism).

It is prescribed through roth, at a dose of 1-2 mg / kg per day. The therapeutic effect appears in 7-10 days.

Like Danazol, this drug is toxic to liver cells and can lead to weight gain and alopecia. In some patients, it causes hemorrhagic cystitis.

They take pomg per day, for 3 months to six months, after the end of treatment, supportive therapy is needed.

While taking the drug, patients are worried about nausea, decreased appetite, vomiting.

Infusion (infusion) of platelets

Splenectomy

Indications - the absence of the expected effect of drug therapy, severe thrombocytopenia and the presence of a risk of dangerous bleeding.

Plasmapheresis

exacerbation of stomach ulcers;
unstoppable bleeding;
hemoglobin level less than 90g / l with iron deficiency anemia;
oncological diseases.

More about plasmapheresis

What is thrombocytopenic purpura and why is it dangerous

Usually, the disease affects children under the age of 10 years, in adults, the disease occurs 2 times less often. The main symptom of this pathology is the appearance of a large number of hemorrhages on the skin and mucous membranes.

Characteristics of the disease

Thrombocytopenia is often diagnosed in patients who suffer from accelerated cell destruction. The method of treatment is selected depending on how much the disease is developed and how much the platelet count has dropped.

Symptoms

In children and adults, the disease manifests itself in a similar way. Since it does not have pronounced signs, for an accurate diagnosis, it is necessary to do a general blood and urine test, as well as undergo some examinations.

The main symptoms of thrombocytopenia are:

the appearance on the skin of hematomas of different sizes; bruising can occur not only as a result of blows, but also completely suddenly, without outside influence;
profuse nosebleeds;
punctate hemorrhages in the upper layers of the epidermis and periodic - in the mucous membranes of the eyes;
frequent dizziness;
increased bleeding of the gums when brushing teeth;
periodic appearance of blood clots in urine and feces, vomiting;
an increase in the size of the spleen (observed in the autoimmune form of the disease);
kidney dysfunction, which develops if the disease is in advanced form.

If a child suffers from thrombocytopenia, there is an increased likelihood of swollen lymph nodes and tenderness during palpation.

Classification and forms

In international medicine, thrombocytopenic purpura is divided into several different forms, depending on what became the primary source of the pathology and determined the clinical picture of the disease.

Classification according to the course and duration of the disease:

Sharp. Most often diagnosed in children under 8 years of age. The exacerbation of the disease disappears when the platelet count rises to normal (this usually takes about 6 months). With this form, relapses are extremely rare, more often complete recovery occurs.
Chronic. Usually diagnosed in adult patients, the duration of the disease is more than 6 months.
Recurrent. Depending on the patient's condition, the platelet level will periodically normalize, then decrease again.

Also, this disease is classified according to the mechanism of occurrence. After conducting examinations, doctors can give patients a specific diagnosis:

Idiopathic form. It is she who is known as Werlhof's disease, arises for no apparent reason (it is almost impossible to establish them).
Isoimmune. It develops if the patient receives frequent blood transfusions.
Transimmune. It occurs in the fetus while it is in the womb. The disease is usually transmitted through antiplatelet antibodies. In most cases, the value of the indicators drops significantly only in the first month of life, after which it normalizes.
Heteroimmune. The antigenic structure of platelets changes due to the fact that they are negatively affected by foreign agents. This form of the disease can develop due to certain viral diseases or under the influence of medications. In the event that the disease does not go away for more than 6 months, it is transferred to an autoimmune form.
Autoimmune. It occurs as a result of various internal pathologies, for example, after lupus erythematosus.

Thrombotic purpura belongs to a separate category. This type of disease is always severe and has an unfavorable prognosis for the patient. Most often, the disease develops in patients aged 30 to 40 years.

The peculiarity of thrombotic purpura is that damaged platelets begin to stick together and clog the vessels, which can provoke ischemia of internal organs. The disease primarily affects the lungs, kidneys, heart and brain.

The acute form of the disease quickly begins to progress, with it, the patient begins to suffer from profuse bleeding, fever, tremors, seizures and visual impairment.

All these symptoms together often lead to coma. According to statistics, this form of the disease is fatal in 60% of cases.

Stages of pathology

The severity of symptoms may differ depending on how much the platelet count has dropped. Based on this indicator, the stages of the disease are distinguished:

Lightweight. With this degree of the disease, platelets decrease slightly. Due to the lack of pronounced signs, the patient will not even guess about the presence of the disease. The only thing that may indicate a pathology is periodic bleeding.
Average. At this stage, a person has rashes all over the body, and can also occur on the mucous membranes.
Heavy. The rash covers most of the body, and gastrointestinal bleeding occurs.

The main causes of the disease

Despite numerous clinical studies and comparison of various factors, leading physicians still cannot say for sure what the pathogenesis of purpura is. In about 45% of cases, doctors cannot identify what triggers the development of the disease.

Today, most experts believe that thrombocytopenia occurs against the background of factors such as:

improper work of the circulatory system;
the formation of benign and malignant neoplasms in the bone marrow;
genetic predisposition;
constant stress and emotional stress;
severe frostbite;
capillary prosthetics operations;
ultraviolet irradiation;
physical damage;
radiation sickness;
long-term use of certain medications, treatment with antibacterial agents and estrogen-based drugs is especially negative.

The risk group includes patients who have suffered diseases such as:

whooping cough;
chicken pox;
measles rubella;
Infectious mononucleosis;
flu.

The likelihood of developing the disease increases in people suffering from spleen dysfunction and those who have inhibited serotonin synthesis.

Diagnostics

In order to choose the optimal treatment method, the physician must take into account all the symptoms and factors in the aggregate. To determine the clinical picture, an external therapeutic examination is performed, anamnesis is taken, and the patient is examined. Looking for:

a general blood test to determine the concentration of platelets;
biochemical analysis of urine;
myelonogram.

For purple, deviations are inherent:

A sharp decrease in the level of platelets.
An increase in the size of these cells and a change in their structure.
Decrease in hemoglobin.
Increased bleeding time.
The presence of antiplatelet antibodies in the patient's blood.

Differential diagnosis is prescribed if there is a suspicion of secondary purpura.

What treatment is used

In medical practice, there are sometimes cases when the disease proceeds in isolation. At the same time, there is mild bleeding, a moderate level of platelet reduction. In such situations, treatment is usually not given. The main task of the patient at this stage is to prevent the progression of the disease.

If the patient is diagnosed with a moderate or severe stage of thrombocytopenia, medications of certain pharmaceutical groups are prescribed.

Also, treatment can be carried out using methods such as:

Plasmapheresis. A procedure that helps clear excess antibodies from the blood.
Transfusion of open erythrocytes and plasma (this method is especially effective for large blood loss).
If the disease is difficult to treat, the patient may be ordered to remove the spleen.

Each of these methods has advantages and disadvantages. The method of therapy should be selected by the attending physician, depending on the individual characteristics of the organism.

Drug therapy

Drug treatment is considered the mainstay of thrombocytopenic purpura. Typically, the patient is prescribed the following drugs:

Corticosteroids. Discharged with profuse hemorrhages, delay autoimmune processes.
Immunosuppressants Medicines of this pharmaceutical group suppress autoimmune reactions.
Means that accelerate blood circulation.
Antiplatelet agents. Drugs that reduce the likelihood of blood clots forming.

If conservative treatment is ineffective, surgery is the only way to save the patient's life.

Nutritional support

Most doctors advise their patients to eat as many foods as possible that help thin the blood and prevent blood clots from forming.

It is necessary to exclude spicy and hot dishes from the diet, due to which the gastric mucosa can be damaged.

To prevent thrombocytopenia from progressing, and the condition to improve, it is necessary to consume:

buckwheat and oatmeal;
corn;
beef liver;
sprouted wheat;
vegetables and fruits;
nuts.

Traditional methods of treatment

The most effective are:

A decoction of viburnum bark. 2 tablespoons of dry bark pour 300 ml of boiling water and put to simmer on a small fire. After the broth boils, turn it off and let it brew for 2 hours. The tool is taken three times a day, 50 ml.
Sesame powder. A small handful of seeds should be crushed and the resulting powder should be taken in a tablespoon 3 times a day (it should be washed down with plenty of water).
Barberry tincture. 20 g of chopped barberry leaves should be poured with 250 ml of water and placed in a dark place for 2 weeks, shaking occasionally. After this time, the medicine will be ready, it is taken three times a day, 5 ml.

Possible complications

If the patient suffers from heavy bleeding, after a few months he may be diagnosed with anemia or iron deficiency anemia. Children under 10 years of age with this ailment often develop stomatitis, gingivitis.

Thrombocytopenic purpura in children and adults photo

Diseases of the skin type affect children and adults with the same frequency. One of these ailments is thrombocytopenic purpura. Photos will be discussed in the article. The disease is caused by a complex nature and a special course. Let's consider how this process takes place in children, how often it forms in adults, and what treatment methods are considered the most effective for eliminating the disease.

Thrombocytopenic purpura what is it

Thrombocytopenic purpura, the photo of which is presented below, is one of the varieties of the hemorrhagic phenomenon - diathesis. The disease is characterized by the phenomenon of a deficiency of red plates of blood - platelets, which is often caused by the influence of immune mechanisms. As signs of the disease are arbitrary effusions of blood into the skin, while they can be multiple or single. Also, the disease is accompanied by an increase in uterine, nasal and other blood flows. If there is a suspicion of the progression of this ailment, the task is to assess the clinical data and other diagnostic information - ELISA, tests, smears and punctures.

This benign pathological disease is accompanied by the fact that there is a deficiency of platelets in the blood, and there is a tendency to increased bleeding and the development of a hemorrhagic symptom. In the presence of an ailment in the peripheral blood system, a downward change in the level of blood plates is observed. Among all hemorrhagic types of diathesis, it is purpura that occurs most often, mainly in children of the preschool age factor. In adults and adolescents, the pathological syndrome is less common and affects mainly women.

Causes of the disease

Often, the manifestation of the disease occurs in the framework of active or passive immunization. Several more factors provoking the formation of the disease are taking medications, X-ray irradiation, exposure to chemicals, surgical interventions, insolation.

The onset of any disease and manifestation is especially acute. The sudden appearance of hemorrhages aggravates the situation and creates a characteristic appearance of the cover for the disease. Against the background of the primary symptoms, additional signs arise - nosebleeds, deterioration of the general condition, pallor of the skin, a sharp increase in the body temperature. If this is an acute process, complete recovery without relapse usually occurs after 1 month. Against the background of the manifestation of the disease, many children are faced with a noticeable increase in lymph nodes. Due to the mild course in about 50% of children, the disease cannot be diagnosed.

If we are talking about a chronic form of the disease, an acute onset is not provided. The first symptoms appear long before acute pronounced symptoms begin. Bleeding occurs in the area of the skin and looks like punctate hemorrhages. They aggravate the overall picture with an exacerbation of the disease. In the first place among the formations is skin purpura - a rash in a child or adult. Hemorrhagic manifestations usually appear abruptly, mainly at night.

In rare situations, “bloody tears” can be identified as a symptom, which is caused by profuse bleeding from the eye area. Usually this phenomenon is observed together with skin formations. The blood flows persistently and abundantly, against the background of this phenomenon, anemia may occur. Gastrointestinal bleeding is less common, but in girls, the process can be accompanied by uterine pathologies and secretions. Rarely, with purpura, hematomas and prolonged blood flow may occur. Children suffering from the disease usually have no complaints, except that a couple of signs can be distinguished.

General state of lethargy and apathy;
increased fatigue;
normal body temperature;
normal size of the spleen and liver;
sharp and severe irritability.

Chronic forms of the disease usually occur in children after 7 years of age. The course is undulating, when remissions and exacerbations alternate.

Thrombocytopenic purpura in children photo

Thrombocytopenic purpura, a photo of which is presented in the article, has several forms and manifestations. The main signs of its presence in young children are hemorrhages and effusions in the area of mucous membranes and under the skin. The color of the hemorrhages ranges from red to green, and the size of the rash on the face and body starts from small petechiae to large blisters and spots.

The manifestation is characterized by the absence of symmetry and the logic of the sequence of education. In children, education does not hurt or bother. When the stage of remission of the clinical type comes, the disease disappears and disappears completely.

Cutaneous hemorrhagic syndrome has several specific and characteristic manifestations.

The severity of the hemorrhagic symptom does not correspond to the traumatic effect. Formations can appear abruptly and unexpectedly.
During the course of childhood illness, polymorphism is observed, in which the variety and size indicators of rashes are different and multifaceted.
Cutaneous hemorrhage has no favorite localization sites. For example, rashes on the hands, body, face, rashes on the legs and other areas of the body may appear.

The most dangerous manifestation of childhood illness is the appearance of hemorrhage in the sclera, which can give rise to the most serious complication - cerebral hemorrhage. The outcome of this course depends on the form and location of the pathology, as well as on the therapeutic measures taken. Speaking about the prevalence of the disease, it can be noted that its frequency ranges from 10 to 125 cases per million population per year.

Thrombocytopenic purpura in children treatment

Before prescribing effective treatment methods, a study of the disease is carried out - a detailed diagnosis. It is possible that a decrease in the number of platelets and the greatest bleeding time will be found in the blood. There is not always a correspondence between the duration of this phenomenon and the degree of thrombocytopenia. The altered properties of blood, changes in hormones in it, as well as other substances act as secondary indicators of the fact of the presence of a disease.

Differential diagnosis

The disease must be differentiated from a number of other manifestations that are similar in their signs and symptomatic indicators.

Acute leukemia - in this case, metaplasia in the bone marrow is manifested.
SLE - for a detailed diagnosis, it is necessary to take into account information from an immunological study.
Thrombocytopathy involves a change in the content and level of platelets in the opposite direction.

Treatment activities for children

Thrombocytopenic purpura, a photo of which is presented in the article, assumes no treatment if the forms of the disease are not severe. With moderate severity, therapeutic interventions are indicated if there is an increased risk of bleeding. Such phenomena are predominantly characteristic of ulcers, hypertension, duodenal ulcer. If the platelet count in the analysis reaches a value<20-30×10^9/л, то терапия назначается в стационарных условиях. Если больной человек страдает от хронической формы проявления заболевания, для которой характерны обильные течения крови и попадание жидкости в жизненно важные элементы, проводится спленэктомия. Ребенок во время геморрагического криза должен проводить время в постели.

If the therapy is pathogenetic, glucocorticosteroids are prescribed at 2 mg / kg of body weight per day by the oral route, immunosuppressants are used (cyclophosphamide, vincristine, if other methods are ineffective). All funds are specifically prescribed by the doctor.
To eliminate the symptoms and features of the manifestation of the disease, human Ig is used, while the child is prescribed a dosage of 1 g per kg of body weight for two days.
Danazol, which has an androgenic effect, is often used, as well as drugs of the interferon group - ROFERON-A, REAFERON, ANTI-A.
The remedies of the symptomatic group are aminocaproic acid, ethamsylate.

Forecast factors

In most situations, the prognosis is favorable. In children, full recovery occurs in 90% of cases, in adults - in 75%. In the acute stage of the process, noticeable complications in the form of a hemorrhagic stroke may occur, while there is a high risk of death. When carrying out treatment, it is necessary that the sick person is constantly under the supervision of a hematologist, while it is important to exclude from the diet of treatment agents that affect the properties of platelet aggregation. These drugs include caffeine, aspirin, barbiturate.

Thrombocytopenic purpura in adults photo

A rash in adults, along with other symptoms, also occurs. The clinical picture is similar to the manifestations of the disease in children. Spots and formations on the skin do not bother a person. The only caveat is that thrombocytopenic purpura (see the photo in the article) among the adult population is 2-3 times more likely to affect the fair sex.

Thrombocytopenic purpura in adults treatment

The medical complex in children and adults has many similarities, and all therapeutic methods are determined by how severe the disease is and how it should last. Treatment consists of conservative therapy, which involves taking medications, as well as the use of surgical methods. Regardless of the manifestation of the disease, bed rest is an important part of recovery.

Drug treatment

Previously, remedies for the treatment of an ailment in children were described, in principle, all of them are suitable for adults, but to simplify the situation and improve the condition, it is recommended to include hormonal drugs in the diet that help to reduce the vascular permeability index and increase blood clotting. If there is multiple rash on the body, as well as other symptoms, the use of several groups of funds is effective.

Prednisolone is applied at 2 mg per kg of body weight per day, subsequently the dosage is reduced. Usually, this therapy gives a tremendous effect, in patients after hormone withdrawal, a relapse occurs.
If the measures taken in adults have not yielded results, immunosuppressants are used. This is imuran, 2-3 mg per kg of body weight per day. The duration of therapy is up to 5 months. Also vincristine 1–2 mg once a week.

Diet for thrombocytopenic purpura

The main requirement, which assumes the possibility of quickly getting rid of the disease, is adherence to a special diet. The patient's diet should be moderately nutritious and complete. Food is served chilled, it should be liquid and contain small portions. The rash, the photo of which was presented in the article, can quickly disappear if you use the right foods.

Food to lower platelets

There is no specific medical menu, but the main rule is to get complete food with proteins and essential vitamins. The benefit lies in the use of several product groups.

Buckwheat porridge;
peanuts and other nuts;
fruits and juices from them;
any vegetables;
corn;
beef liver;
oat flakes;
wheat and products from it.

What to exclude from the diet

spicy and salty foods;
high fat foods;
food with impurities and flavorings;
hot spices and sauces;
restaurant food and fast food products.

Do you know the disease thrombocytopenic purpura? Did the photo help with the definition of symptoms? Leave your feedback for everyone on the forum!

Thrombocytopenic purpura

Thrombocytopenic purpura is a type of hemorrhagic diathesis characterized by a deficiency of red platelets - platelets, often caused by immune mechanisms... Signs of thrombocytopenic purpura are spontaneous, multiple, polymorphic hemorrhages in the skin and mucous membranes, as well as nasal, gingival, uterine and other bleeding. If thrombocytopenic purpura is suspected, anamnestic and clinical data, indicators of a general blood test, coagulogram, ELISA, microscopy of blood smears, bone marrow puncture are evaluated. V medicinal purposes patients are prescribed corticosteroid, hemostatic drugs, cytostatic therapy, splenectomy is performed.

Thrombocytopenic purpura

Thrombocytopenic purpura (Werlhof's disease, benign thrombocytopenia) is a hematological pathology characterized by a quantitative deficiency of platelets in the blood, accompanied by a tendency to bleeding, the development of hemorrhagic syndrome. With thrombocytopenic purpura, the level of platelets in peripheral blood drops significantly below physiological - 150x10 9 / l with a normal or slightly increased number of megakaryocytes in the bone marrow. In terms of the frequency of occurrence, thrombocytopenic purpura ranks first among other hemorrhagic diathesis. The disease usually manifests in childhood (with a peak in the early and preschool period). In adolescents and adults, pathology is 2-3 times more likely to be detected among females.

The classification of thrombocytopenic purpura takes into account its etiological, pathogenetic and clinical features... There are several options - idiopathic (Werlhof disease), iso-, trans-, hetero- and autoimmune thrombocytopenic purpura, Werlhof symptom complex (symptomatic thrombocytopenia).

Acute, chronic and recurrent forms are isolated along the flow. The acute form is more typical for childhood, lasts up to 6 months with the normalization of the level of platelets in the blood, has no relapses. The chronic form lasts more than 6 months, is more common in adult patients; recurrent - has a cyclical course with repetitions of episodes of thrombocytopenia after the normalization of platelet levels.

Causes of thrombocytopenic purpura

In 45% of cases, idiopathic thrombocytopenic purpura occurs, developing spontaneously, for no apparent reason. In 40% of cases, thrombocytopenia is preceded by various infectious diseases(viral or bacterial), transferred about 2-3 weeks before. In most cases, these are infections of the upper respiratory tract of nonspecific genesis, in 20% - specific (chickenpox, measles, rubella, parotitis, infectious mononucleosis, whooping cough). Thrombocytopenic purpura can complicate the course of malaria, typhoid fever, leishmaniasis, septic endocarditis. Sometimes thrombocytopenic purpura manifests itself against the background of immunization - active (vaccination) or passive (administration of γ - globulin). Thrombocytopenic purpura can be triggered by taking medications (barbiturates, estrogens, arsenic, mercury), prolonged exposure to X-rays (radioactive isotopes), extensive surgery, trauma, excessive insolation. Familial cases of the disease have been reported.

Most variants of thrombocytopenic purpura are immune in nature and are associated with the production of antiplatelet antibodies (IgG). The formation of immune complexes on the surface of platelets leads to the rapid destruction of platelets, a decrease in their life expectancy to several hours instead of 7-10 days in the norm.

The isoimmune form of thrombocytopenic purpura can be caused by the entry into the blood of "foreign" platelets during repeated transfusions of blood or platelet mass, as well as antigenic incompatibility of the mother's and fetal platelets. The heteroimmune form develops when the antigenic structure of platelets is damaged by various agents (viruses, medicines). The autoimmune variant of thrombocytopenic purpura is caused by the appearance of antibodies against their own unchanged platelet antigens and is usually combined with other diseases of the same genesis (SLE, autoimmune hemolytic anemia). The development of transimmune thrombocytopenia in newborns is triggered by the antiplatelet autoantibodies of a mother with thrombocytopenic purpura passing through the placenta.

Deficiency of platelets in thrombocytopenic purpura may be associated with functional damage to megakaryocytes, a violation of the process of lacing the red platelets. For example, the Verlhof symptom complex is due to the ineffectiveness of hematopoiesis in anemia (B-12 deficiency, aplastic), acute and chronic leukemia, systemic diseases of the hematopoietic organs (reticulosis), bone marrow metastases of malignant tumors.

With thrombocytopenic purpura, there is a violation of the formation of thromboplastin and serotonin, a decrease in contractility and an increase in the permeability of the capillary wall. Associated with this are the lengthening of the bleeding time, the violation of the processes of thrombus formation and retraction of the blood clot. With hemorrhagic exacerbations, the number of platelets decreases down to single cells in the preparation, during the period of remission it is restored to a level below normal.

Thrombocytopenic Purpura Symptoms

Thrombocytopenic purpura is clinically manifested when the platelet count falls below 50x10 9 / l, usually 2-3 weeks after exposure to the etiological factor. Bleeding is characteristic of the petechial-spotted (bruised) type. In patients with thrombocytopenic purpura, painless multiple hemorrhages appear under the skin, in the mucous membranes ("dry" version), as well as bleeding ("wet" version). They develop spontaneously (often at night) and their severity does not correspond to the strength of the traumatic effect.

Hemorrhagic rashes are polymorphic (from minor petechiae and ecchymosis to large bruises and bruises) and polychromic (from bright purple-blue to pale yellow-green, depending on the time of occurrence). Most often, hemorrhages occur on the front surface of the trunk and limbs, rarely - in the face and neck. Hemorrhages are also determined on the mucous membrane of the tonsils, soft and hard palate, conjunctiva and retina, eardrum, in adipose tissue, parenchymal organs, serous membranes of the brain.

Pathognomonic intense bleeding- nasal and gingival, bleeding after tooth extraction and tonsillectomy. Hemoptysis, bloody vomiting and diarrhea, blood in the urine may appear. In women, uterine bleeding usually prevails in the form of menorrhagias and metrorrhagias, as well as ovulatory bleeding v abdominal cavity with symptoms ectopic pregnancy... Immediately before menstruation, skin hemorrhagic elements, nose and other bleeding appear. Body temperature remains normal, tachycardia is possible. With thrombocytopenic purpura, there is moderate splenomegaly. With profuse bleeding, anemia of internal organs develops, hyperplasia of the red bone marrow and megakaryocytes.

The drug form manifests itself shortly after administration medicinal product, lasts from 1 week to 3 months with spontaneous recovery. Radiation thrombocytopenic purpura is severe hemorrhagic diathesis with the transition of the bone marrow to the hypo- and aplastic state. The infantile form (in children under 2 years of age) has an acute onset, severe, often chronic, and severe thrombocytopenia (9 / L).

During thrombocytopenic purpura, periods of hemorrhagic crisis, clinical and clinical-hematological remission are detected. With a hemorrhagic crisis, bleeding and laboratory changes are pronounced; during the period of clinical remission against the background of thrombocytopenia, hemorrhages do not appear. At complete remission there are no bleeding and laboratory changes. With thrombocytopenic purpura with large blood loss, acute post-hemorrhagic anemia is observed, with prolonged chronic form - chronic Iron-deficiency anemia.

The most formidable complication - cerebral hemorrhage develops suddenly and progresses rapidly, accompanied by dizziness, headache, vomiting, convulsions, neurological disorders.

Diagnosis of thrombocytopenic purpura

The diagnosis of thrombocytopenic purpura is established by a hematologist, taking into account the history, characteristics of the course and results laboratory research (clinical analysis blood and urine, coagulogram, ELISA, microscopy of blood smears, bone marrow puncture).

Thrombocytopenic purpura is indicated a sharp decline the number of platelets in the blood (9 / L), an increase in bleeding time (> 30 min.), prothrombin time and aPTT, a decrease in the degree or absence of clot retraction. The number of leukocytes is usually within the normal range, anemia appears with significant blood loss. At the height of the hemorrhagic crisis, positive endothelial tests (pinch, tourniquet, prick) are detected. In a blood smear, an increase in the size and a decrease in the granularity of platelets is determined. In preparations of red bone marrow, normal or increased amount megakaryocytes, presence of immature forms, lacing of platelets in small points. The autoimmune nature of purpura is confirmed by the presence of antiplatelet antibodies in the blood.

Thrombocytopenic purpura is differentiated from aplastic or infiltrative processes of the bone marrow, acute leukemia, thrombocytopathies, SLE, hemophilia, hemorrhagic vasculitis, hypo- and dysfibrinogenemia, juvenile uterine bleeding.

Treatment and prognosis of thrombocytopenic purpura

In thrombocytopenic purpura with isolated thrombocytopenia (platelets> 50x10 9 / l) without hemorrhagic syndrome, treatment is not performed; with moderate thrombocytopenia (30-50 x10 9 / l) drug therapy shown in case high risk development of bleeding ( arterial hypertension, peptic ulcer stomach and duodenum 12). At a platelet level of 9 / l, treatment is carried out without additional indications in a hospital setting.

Bleeding is stopped by the introduction of hemostatic drugs, a hemostatic sponge is applied topically. To curb immune reactions and reduce vascular permeability, corticosteroids are prescribed in a lower dose; hyperimmune globulins. With large blood loss, transfusion of plasma and washed erythrocytes is possible. Platelet infusions are not indicated for thrombocytopenic purpura.

In sick chronic form with relapses of heavy bleeding and hemorrhages in vital organs, splenectomy is performed. Perhaps the appointment of immunosuppressants (cytostatics). Treatment of thrombocytopenic purpura, if necessary, should be combined with therapy for the underlying disease.

In most cases, the prognosis of thrombocytopenic purpura is very favorable, complete recovery is possible in 75% of cases (in children - in 90%). Complications (eg, hemorrhagic stroke) occur in the acute stage, posing a risk of death. With thrombocytopenic purpura, constant supervision of a hematologist is required, drugs that affect the aggregation properties of platelets (acetylsalicylic acid, caffeine, barbiturates) are excluded, food allergens, caution is shown when vaccinating children, insolation is limited.

Thrombocytopenic purpura (Werlhof disease): symptoms and treatment

Thrombocytopenic purpura (Verlhof's disease) - the main symptoms:

Headache
Weakness
Abdominal pain
Dizziness
Elevated temperature
Nausea
Loss of appetite
Chest pain
Vomit
Spleen enlargement
Increased fatigue
Bleeding gums
Nosebleeds
Fever
Eye hemorrhage
Visual impairment
Subcutaneous hemorrhage
Hematomas
Uterine bleeding

Thrombocytopenic purpura or Werlhof's disease is a disease that occurs against the background of a decrease in the number of platelets and their pathological tendency to stick together, and is characterized by the appearance of multiple hemorrhages on the surface of the skin and mucous membranes. The disease belongs to the group of hemorrhagic diathesis, is quite rare (according to statistics, 10–100 people get sick with it a year). It was first described in 1735 by the famous German physician Paul Werlhof, after whom it got its name. Most often, everything manifests itself before the age of 10 years, while it affects both sexes with the same frequency, and if we talk about statistics among adults (after 10 years of age), then women get sick twice as often as men.

Etiology of the disease

In most cases, it is impossible to determine the causes of thrombocytopenic purpura, although scientists were able to find out that genetic defects do not play a major role in its development. Sometimes it occurs against the background of hereditary pathologies associated with a disruption in the production of thrombocytopoietins in the body or a decrease in enzymes included in the Krebs cycle, but these are isolated cases.

To the most probable reasons that can cause purpura, doctors refer to:

malfunctions of the hematopoietic system caused by the development of aplastic anemia;
tumor lesions of the bone marrow;
the harmful effect of radiation on the human body, which causes a violation of myelopoiesis - the process of development and maturation shaped elements blood;
a past bacterial or viral infection (according to statistics, in 40% of cases, Verlhof's disease occurs precisely because of this);
vascular replacement surgery that could cause mechanical damage platelets;
pathological reaction of the body to the introduction of gamma globulin;
application of some contraceptive drugs;
chemotherapy for the treatment of oncology.

Sometimes the cause of the development of the disease in adults is collagenosis of an autoimmune nature, prolonged blood stagnation or pregnancy (this fact has been confirmed by a few scientists and doctors, therefore it is rarely mentioned).

The mechanism of development of Werlhof's disease

The onset of the disease is characterized by a rapid decrease in the number of platelet components in relation to the total blood volume, which leads to a violation of its coagulability. This process is accompanied by a violation of the trophism (nutrition) of blood vessels, dystrophy vascular endothelium and an increase in the permeability of the vascular walls in relation to erythrocytes. The course of the disease is aggravated by the fact that during its development work is disrupted immune system, which begins to produce antiplatelet immunoglobulins that cause lysis (destruction) of platelets. It should be said that the term idiopathic thrombocytopenic purpura means that all of the above processes are developing rapidly, but from the foregoing it can be concluded that thrombocytopenia is the basis of all pathogenetic processes, which causes vascular dystrophy and becomes the cause of the development of prolonged hemorrhagic hemorrhages.

Clinical picture and types of disease

Depending on the presence (and sometimes absence) of the reasons causing the development of the pathological process in human body, there is idiopathic (true), autoimmune and thrombotic thrombocytopenic purpura.

Doctors have found that Werlhof's disease can exist in acute and chronic forms, while the acute form is most often diagnosed in children. preschool age, and chronic - in adults aged 20 or 50 years. All types of the disease are accompanied by approximately the same symptoms, the difference between which is characterized mainly by the intensity of their manifestation.

Idiopathic thrombocytopenic purpura, as mentioned above, is characterized by spontaneous, acute development, as well as the lack of an opportunity to find out its reasons.
Thrombotic thrombocytopenic purpura is considered the most dangerous manifestation of the disease, because it is characterized by a malignant course, which, as a rule, leads to the death of the patient. It is accompanied by the appearance of hyaline blood clots in the vessels, which completely block the blood flow, and has several symptoms characteristic only of it: fever, neurosis, renal failure (in fact, the patient's death mainly occurs due to the failure of the kidneys).
Autoimmune thrombocytopenic purpura is essentially a type of idiopathic purpura, the reasons for the development of which are still not clear. It is characterized by the fact that the body begins to vigorously produce antibodies against its platelets, thereby significantly reducing their concentration in the blood, and pathological process, as a rule, is chronic.

The main symptoms of the disease

Werlhof's disease, as this disease of hemorrhagic genesis, is primarily accompanied by the presence of various vascular pathologies, which are characterized by the rapid appearance of small (in the early stages) or large subcutaneous hematomas, as well as cutaneous hemorrhages (punctate hemorrhages in the upper layers of the skin). Hematomas can occur spontaneously or as a result of bruises, their number increases if the affected area of the skin (for example, a limb) is briefly tightened with a rubber band. A clinical blood test shows a too low platelet mass. TO frequent symptoms include hemorrhages affecting the mucous membranes of the eyes, the fundus itself and even the brain, which occur without previous symptoms, progress rapidly and are accompanied by dizziness, sometimes nausea and vomiting.

In addition to the above external symptoms thrombotic thrombocytopenic purpura is characterized by low content hemoglobin in the blood, and, as a result, the appearance of symptoms of iron deficiency anemia. The patient's body temperature rises significantly against the background of the appearance of a "hemorrhagic rash", severe pains in the abdomen and hematuria appear (evidence of impaired renal function).

In contrast to thrombotic purpura, autoimmune purpura is also accompanied by a slight enlargement of the spleen, which plays a very important role in the process of platelet formation.

If we talk about how thrombocytopenic purpura proceeds in children, then it is often accompanied by profuse nose and uterine bleeding in boys and girls, respectively.

Diagnostics

The main methods for diagnosing the disease include an external therapeutic examination of the skin, a general blood test to determine the concentration of platelets, a biochemical study of urine, an analysis to determine the intensity of myelopoiesis processes - myelogram. In addition, doctors pay attention to the increased frequency of nose or uterine bleeding, which are considered "indirect" symptoms of the development of the pathological process.

It is worth saying that the rapid development of idiopathic thrombocytopenic purpura in children is diagnosed solely on the basis of a visual examination of the skin (laboratory tests to confirm the diagnosis in this case are not necessary, that is, treatment can be started immediately).

How to treat?

It is necessary to treat the disease (especially in advanced cases) in a hospital setting, because the first task of doctors is to stop the processes of destruction of platelets and increase their concentration in the blood.

Drug treatment begins with the elimination of the symptoms of the disease - local (skin) and general bleeding, for this purpose, drugs with a hemostatic effect ("Ascorutin", "Thrombin") are used. Subsequently, the patient is prescribed glucocorticoids ("Prednisolone") and immunoglobulins. Without positive effect from drug therapy, doctors prescribe surgical treatment, in the form of splenectomy - removal of the spleen. The method is used in extremely rare cases with the threat of extensive cerebral hemorrhage.

If possible (with a slow course of the pathological process), the patient is prescribed several sessions of plasmapheresis (it helps to clear the blood of antibodies to platelets). A transfusion (infusion) of platelets from a donor is also possible.

Given that thrombocytopenic purpura in children is not uncommon, the responsibility for it successful treatment lies with the parents, who should regularly show the child to the doctor and accustom him to healthy way life (hardening, physical activity and proper nutrition will significantly reduce the likelihood of a relapse).

Werlhof disease is very serious pathology the circulatory system, which in some cases can cause the death of the patient, however, with high-quality and timely treatment, the prognosis for most patients will be favorable.

If you think that you have thrombocytopenic purpura (Werlhof's disease) and symptoms characteristic of this disease, then a hematologist can help you.

We also suggest using our online disease diagnostics service, which, based on the entered symptoms, selects probable diseases.

Often, a person can form spots on the surface of the skin, but it is rare who can attribute such manifestations to a violation of the composition of the blood. This disease is called thrombocytopenic purpura and is endowed with certain characteristics.

Causes of pathology

Thrombocytopenic purpura, or as it is also called, Werlhof's disease is a disease that is directly related to a change in the quantitative presence of platelets in the blood. Often, these components can stick together, which also leads to a decrease in their number. Such a process of the disease entails the occurrence of numerous hemorrhages, which can form not only on top layer epidermis, but also on the mucous membranes.

If we talk about the factors influencing the onset and development of pathology, the exact cause of the disease has not yet been identified, but in some cases it can be influenced by a hereditary factor. Additional and less common causes of the disease include:

negative changes in the performance of the circulatory system;
tumor neoplasms in the brain area;
outside influence external environment With increased content radioactive substances;
the presence of an infection in the body of a bacterial or viral nature.

Some medical specialists argue that the appearance of such changes (Werlhof disease) can be influenced by the use of certain contraceptive drugs, as well as the use of chemotherapy to combat oncological diseases... Such a disease most often occurs in childhood; in more mature patients, pathology can be detected mainly in representatives of the weaker half of humanity.

Symptomatic symptoms that appear

As mentioned earlier, the symptomatic signs of this disease begin to manifest themselves immediately after the level of platelets in the blood begins to noticeably decrease. The main visible symptom pathology is the manifestation of rashes on the skin and mucous membranes. They can be of various depths and sizes, while the painfulness of their manifestation can also be different in thrombocytopenic purpura, the symptoms of which may not only have the nature of rashes.

Rashes of this disease can differ in certain characteristics:

Rash characteristic	Varieties of manifestation	Features of manifestation
In appearance	single; strip-like (single manifestations are collected in a so-called group); multiple - represent large spots or long stripes.	The color features correspond to the name, after the smudges begin to heal, they change their color to yellow-green.
By etymology	The rash may be dry, moist (and may be accompanied by minor bleeding, especially at night).	The occurrence is not associated with the result of injury or mechanical influence. I can form on the bottom and upper limbs, are less common on the neck and face. Relatively often, their manifestation is carried out on the mucous membranes, as well as in those places where it is almost impossible to conduct research without using special devices, for example, on the eardrum, the brain membrane, adipose tissue or the surface of internal organs.

Bleeding can often be a symptom of the disease. The form in this case can be as varied as its intensity. The main forms of the disease include:

nasal;
gum;
in combination with phlegm when coughing;
in urine or vomit;
with bowel movements.

It is important to consider other possible symptoms thrombocytopenic purpura. Hemorrhages can also be in the area of internal organs, but such manifestations of the disease are much more serious and have a character of pronounced pain. A change in body temperature with such deviations is not observed, in some cases, a manifestation of a rapid heartbeat is possible. In childhood, when examining a patient, an increase in lymph nodes is revealed, accompanied by pain. With Werlhof's disease, symptoms can be expressed only in relation to laboratory manifestations.

Features of the course of the disease

The process of the course of the disease directly depends on what caused its occurrence. If the formation of pathology was influenced by the effect of drugs, then this form of the disease does not require the use of drugs and can eliminate itself in a maximum of several months. When influenced radiation exposure the patient has a more severe manifestation of the disease, and general state the patient is characterized as severe. In childhood, Werlhof's disease occurs instantly and is relatively difficult. Its transition to a chronic form with a minimal presence of platelets in the blood is not excluded.

Thrombocytopenia has several stages:

hemorrhagic crisis - symptomatic signs are pronounced, when studying blood, a reduced number of platelets present is observed;
clinical remission - symptoms can be eliminated, however, clinical pathological processes take place;
clinical and hematological remission - characterized by the restoration of all abnormal indicators.

At certain stages of the course of werlhof disease in patients, a febrile state may be detected, the manifestation of seizures is relatively lower limbs, in a more complex form, the spread of cerebral coma, the presence of pain in the lower abdomen, as well as the presence of pain in the articular joints are possible.

Diagnosis and Treatment Methods

In order for the attending physician to be able to accurately establish a diagnosis, he needs to take into account all the symptomatic signs and factors that appear that provoke the development of pathology. In this case, when conducting a survey, you should pay attention to the time period in relation to external influence factors, the manifestation of the disease on the part of the patient's relatives, as well as the possible presence of bleeding, both external and internal organs.

After that, a number of necessary laboratory tests are carried out, based on the results of which a diagnosis is made by a hematologist.

The way in which thrombocytopenia will be eliminated is established depending on how difficult and painful the pathological process of Verlhof is itself, the treatment of which has several options. Depending on this, either the traditional method of treating the disease with drugs is used, or through surgical intervention... Whatever the form of pathology and the nature of its manifestation, the process of treating the disease is carried out only within the hospital with the obligatory observance of bed rest. Nutrition deserves special attention, since it is extremely necessary to follow the diet during the period of this disease, the diet should be complete and moderately high in calories. Servings should be small, preferably liquid.

As for the use of drugs in the diagnosis of Werlhof's disease, hormonal drugs deserve special attention. They help to improve the process of blood clotting and are used, as a rule, for extensive damage to the skin during this disease. The most popular is Prednisolone, which is initially taken in high dosages. The effectiveness of the treatment of the disease is noticed already after the first two weeks of treatment, after which the volume medicinal product decreases. In the future, the patient needs to receive special vitamin complexes, and to stop bleeding and prevent them, it is suitable to take medicinal fees, which include nettle, rose hips and yarrow.

In certain situations, specialists have to resort to such a form of treatment for the disease as a blood transfusion. This is extremely necessary when diagnosing a severe form of the disease or in order to stop active and extensive bleeding. If the above methods are ineffective in relation to the diagnosed disease, then the patient is assigned surgical removal spleen.

This disease does not exclude the manifestation serious complications, the main of which is considered a stroke or hemorrhage in the region of the brain. Such a process is formed instantly, while disturbing consciousness, provoking the development of headache, vomiting and numerous neurological disorders. In the case of major bleeding, it is possible to subsequently diagnose the patient with anemia, and in a chronic course - such a disease as anemia of iron deficiency etymology.

Very rarely, when purpura disease can be fatal, as a rule, positive dynamics and complete cure of the patient are observed. All possible fears take place only in the acute form of the disease.

It is important to remember about preventive measures regarding this disease. In this case, it is recommended to monitor the diet so that only those foods that have minimal allergenic properties are included in the diet. It is also important to consider the impact of the drugs taken. Patients should control the influence of sunlight and especially ultraviolet radiation on the existing disease.

It is especially important to refrain from contact with infectious patients.

As for pediatric patients, in this case it is important to regularly show the child to medical specialists for timely examination and possible detection of the disease. The child should be taught to a healthy lifestyle, mainly hardening, physical activity... It is especially important to monitor the child's nutritional process, even if the disease has not yet been diagnosed.

Thus, a disease such as thrombocytopenic purpura in adults is a serious and problematic process. He demands compulsory treatment and timely diagnosis, since at the initial stages of its development, the possibility of bleeding is reduced, especially related to internal organs. To date, the disease is not fully understood, however, medical specialists offer several options for its elimination and prevention at once.

In contact with

A type of hemorrhagic diathesis, characterized by a deficiency of red platelets - platelets, often caused by immune mechanisms. Signs of thrombocytopenic purpura are spontaneous, multiple, polymorphic hemorrhages in the skin and mucous membranes, as well as nasal, gingival, uterine and other bleeding. If thrombocytopenic purpura is suspected, anamnestic and clinical data, indicators of a general blood test, coagulogram, ELISA, microscopy of blood smears, bone marrow puncture are evaluated. For therapeutic purposes, patients are prescribed corticosteroid, hemostatic drugs, cytostatic therapy, and splenectomy is performed.

General information

Thrombocytopenic purpura (Werlhof's disease, benign thrombocytopenia) is a hematological pathology characterized by a quantitative deficiency of platelets in the blood, accompanied by a tendency to bleeding, the development of hemorrhagic syndrome. With thrombocytopenic purpura, the level of platelets in the peripheral blood drops significantly below the physiological level - 150x10 9 / l with a normal or slightly increased number of megakaryocytes in the bone marrow. In terms of the frequency of occurrence, thrombocytopenic purpura ranks first among other hemorrhagic diathesis. The disease usually manifests in childhood (with a peak in the early and preschool period). In adolescents and adults, pathology is 2-3 times more likely to be detected among females.

In the classification of thrombocytopenic purpura, its etiological, pathogenetic and clinical features are taken into account. There are several options - idiopathic (Werlhof disease), iso-, trans-, hetero- and autoimmune thrombocytopenic purpura, Werlhof symptom complex (symptomatic thrombocytopenia).

Causes of thrombocytopenic purpura

In 45% of cases, idiopathic thrombocytopenic purpura occurs, developing spontaneously, for no apparent reason. In 40% of cases, thrombocytopenia is preceded by various infectious diseases (viral or bacterial), transferred about 2-3 weeks before. In most cases, these are infections of the upper respiratory tract of nonspecific genesis, in 20% - specific (chickenpox, measles, rubella, mumps, infectious mononucleosis, whooping cough). Thrombocytopenic purpura can complicate the course of malaria, typhoid fever, leishmaniasis, septic endocarditis. Sometimes thrombocytopenic purpura manifests itself against the background of immunization - active (vaccination) or passive (administration of γ - globulin). Thrombocytopenic purpura can be triggered by taking medications (barbiturates, estrogens, arsenic, mercury), prolonged exposure to X-rays (radioactive isotopes), extensive surgery, trauma, excessive insolation. Familial cases of the disease have been reported.

Deficiency of platelets in thrombocytopenic purpura may be associated with functional damage to megakaryocytes, a violation of the process of lacing the red platelets. For example, Verlhof's symptom complex is due to the ineffectiveness of hematopoiesis in anemia (B-12 deficiency, aplastic), acute and chronic leukemia, systemic diseases of the hematopoietic organs (reticulosis), bone marrow metastases of malignant tumors.

Thrombocytopenic Purpura Symptoms

Hemorrhagic rashes are polymorphic (from minor petechiae and ecchymosis to large bruises and bruises) and polychromic (from bright purple-blue to pale yellow-green, depending on the time of occurrence). Most often, hemorrhages occur on the front surface of the trunk and limbs, rarely - in the face and neck. Hemorrhages are also determined on the mucous membrane of the tonsils, soft and hard palate, conjunctiva and retina, tympanic membrane, in adipose tissue, parenchymal organs, serous membranes of the brain.

Intense bleeding is pathognomonic - nasal and gingival, bleeding after tooth extraction and tonsillectomy. Hemoptysis, bloody vomiting and diarrhea, blood in the urine may appear. In women, uterine bleeding usually prevails in the form of menorrhagias and metrorrhagias, as well as ovulatory bleeding into the abdominal cavity with symptoms of ectopic pregnancy. Immediately before menstruation, skin hemorrhagic elements, nose and other bleeding appear. Body temperature remains normal, tachycardia is possible. With thrombocytopenic purpura, there is moderate splenomegaly. With profuse bleeding, anemia of internal organs develops, hyperplasia of the red bone marrow and megakaryocytes.

The drug form manifests itself shortly after taking the drug, lasts from 1 week to 3 months with spontaneous recovery. Radiation thrombocytopenic purpura is characterized by severe hemorrhagic diathesis with the transition of the bone marrow to a hypo- and aplastic state. The infantile form (in children under 2 years of age) has an acute onset, severe, often chronic, and severe thrombocytopenia (9 / L).

During thrombocytopenic purpura, periods of hemorrhagic crisis, clinical and clinical-hematological remission are detected. With a hemorrhagic crisis, bleeding and laboratory changes are pronounced; during the period of clinical remission against the background of thrombocytopenia, hemorrhages do not appear. With complete remission, there is no bleeding or laboratory changes. With thrombocytopenic purpura with large blood loss, acute post-hemorrhagic anemia is observed, with long-term chronic form, chronic iron deficiency anemia.

The most formidable complication - cerebral hemorrhage develops suddenly and progresses rapidly, accompanied by dizziness, headache, vomiting, convulsions, neurological disorders.

Diagnosis of thrombocytopenic purpura

The diagnosis of thrombocytopenic purpura is established by a hematologist, taking into account the history, course characteristics and results of laboratory tests (clinical blood and urine analysis, coagulogram, ELISA, microscopy of blood smears, bone marrow puncture).

Thrombocytopenic purpura is indicated by a sharp decrease in the number of platelets in the blood (9 / L), an increase in bleeding time (> 30 minutes), prothrombin time and APTT, a decrease in the degree or absence of clot retraction. The number of leukocytes is usually within the normal range, anemia appears with significant blood loss. At the height of the hemorrhagic crisis, positive endothelial tests (pinch, tourniquet, prick) are detected. In a blood smear, an increase in the size and a decrease in the granularity of platelets is determined. In preparations of red bone marrow, a normal or increased number of megakaryocytes, the presence of immature forms, and lacing of platelets in small points are found. The autoimmune nature of purpura is confirmed by the presence of antiplatelet antibodies in the blood.

Thrombocytopenic purpura is differentiated from aplastic or infiltrative processes of the bone marrow, acute leukemia, thrombocytopathy, SLE, hemophilia, hemorrhagic vasculitis, hypo- and dysfibrinogenemia, juvenile uterine bleeding.

Treatment and prognosis of thrombocytopenic purpura

In thrombocytopenic purpura with isolated thrombocytopenia (platelets> 50x10 9 / l) without hemorrhagic syndrome, treatment is not performed; with moderate thrombocytopenia (30-50 x10 9 / l), drug therapy is indicated in case of an increased risk of bleeding (arterial hypertension, gastric ulcer and duodenal ulcer). At a platelet level of 9 / l, treatment is carried out without additional indications in a hospital setting.

In patients with a chronic form with relapses of profuse bleeding and hemorrhages in vital organs, splenectomy is performed. Perhaps the appointment of immunosuppressants (cytostatics). Treatment of thrombocytopenic purpura, if necessary, should be combined with therapy for the underlying disease.

In most cases, the prognosis of thrombocytopenic purpura is very favorable, complete recovery is possible in 75% of cases (in children - in 90%). Complications (eg, hemorrhagic stroke) occur in the acute stage, posing a risk of death. With thrombocytopenic purpura, constant supervision of a hematologist is required, drugs that affect the aggregation properties of platelets (acetylsalicylic acid, caffeine, barbiturates), food allergens are excluded, caution is shown when vaccinating children, insolation is limited.