Platelet purpura in children. Thrombocytopenic purpura (Werlhof disease)

Often, a person can form spots on the surface of the skin, but rarely, anyone can attribute such manifestations to a violation of the blood composition. 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 disease process entails the occurrence of numerous hemorrhages, which can form not only on the upper layer of the epidermis, but also on the mucous membranes.

If we talk about the factors affecting the occurrence and development of pathology, then 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 change in the performance of the circulatory system;
tumor neoplasms in the brain area;
impact from the external environment with a high content of radioactive substances;
the presence of an infection in the body of a bacterial or viral nature.

Some medical experts argue that the appearance of such changes (Werlhof's disease) may be influenced by the use of certain contraceptive drugs, as well as the use of chemotherapy to combat cancer. 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.

Presenting symptomatic signs

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

The rashes of this disease can differ in certain characteristics:

Characteristics of the rash	Varieties of manifestation	Features of manifestation
By appearance	single; striped (single manifestations are collected in the so-called group); multiple - are 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, wet (and may be accompanied by minor bleeding, especially at night).	The occurrence is not associated with the result of injury or mechanical influence. Can form on the lower and upper limbs, 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 practically impossible to conduct a study without the use of special devices, for example, on the eardrums, the meninges, adipose tissue or the surface of internal organs.

Often, bleeding can be a symptom of the disease. The form in this case can be as diverse as its intensity. The main forms of the disease include:

nasal;
gingival;
in combination with sputum when coughing;
in urine or vomit;
during bowel movements.

It is important to consider other possible symptoms of thrombocytopenic purpura. Hemorrhages can also be in the area of the internal organs, however, such manifestations of the disease are much more serious and have the character of pronounced pain. A change in body temperature with such deviations is not observed, in some cases, palpitations may occur. In childhood, when examining a patient, an increase in lymph nodes is detected, accompanied by pain. With Werlhof's disease, symptoms can only be expressed 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 it. If the formation of pathology was influenced by the effects of drugs, then this form of the disease does not require the use of drugs and can resolve itself after a maximum of a few months. Under the influence of radiation exposure, the patient has a more severe manifestation of the disease, and the general condition of 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's disease, patients may have a feverish condition, manifestation of convulsions in relation to the lower extremities, in a more complex form, the spread of cerebral coma, the presence of pain in the lower abdomen, and the presence of pain in the articular joints are possible.

Methods of diagnosis and treatment

In order for the attending physician to be able to accurately establish the diagnosis, he needs to take into account all the manifesting symptomatic signs and factors that provoke the development of pathology. In this case, when conducting a survey, attention should be paid to the time period relative to the external influence of 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 from the 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 set depending on how difficult and painful the pathological process of Werlhof itself is, the treatment of which has several options. Depending on this, either the traditional method of treating the disease with the help of drugs, or through surgical intervention, is used. Whatever the form of the 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 a diet during this illness, the diet should be complete and moderately high-calorie. Servings should be small, preferably in liquid form.

As for the use of drugs in the diagnosis of Werlhof's disease, hormonal preparations 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 large dosages. The effectiveness of the treatment of the disease is noticed already after the first two weeks of treatment, after which the volume of the drug decreases. In the future, the patient needs to take special vitamin complexes, and to stop bleeding and prevent them, it is suitable to take medicinal preparations, which include nettle, rosehip and yarrow.

In certain situations, specialists have to resort to a form of treatment for the disease, such 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 prescribed surgical removal of the spleen.

This disease does not exclude the manifestation of serious complications, the main of which is a stroke or hemorrhage in the brain area. Such a process is formed instantly, while disturbing consciousness, provokes the development of headaches, vomiting and numerous neurological disorders. In the case of major bleeding, a subsequent diagnosis of an anemia disease in a patient is possible, and in a chronic course, a disease such as anemia of iron deficiency etymology.

Very rarely, when purpura disease can be fatal, as a rule, there is a positive trend and a complete cure for the patient. 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 nutrition so that only those foods that have minimally allergenic properties are included in the diet. It is important to take into account the impact of the medications taken. Patients should monitor the effect 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. It is necessary to accustom the child to a healthy lifestyle, mainly hardening, physical activity. It is especially important to control the child's nutrition 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. It requires mandatory treatment and timely diagnosis, since at the initial stages of its development, the possibility of bleeding is reduced, especially those related to internal organs. To date, the disease has not been fully studied, however, medical specialists offer several options for its elimination and prevention, including.

In contact with

Idiopathic thrombocytopenic purpura (ITP) - a disease that is characterized by a significant decrease in the level of platelets in the blood, which usually leads to hemorrhagic syndrome, that is, increased bleeding. The term "Werlhof's disease" is also used in honor of the doctor who first described the disease. It is also proposed to use the term "primary immune thrombocytopenia".

In the patient's body, antibodies are formed to his own platelets, the rate of destruction of blood platelets increases several times, and there is a deficiency of these cells. It is ITP that is the most common cause of hemorrhagic syndrome in patients observed by hematologists.

There are acute and chronic forms of ITP. Acute forms (constituting the majority of cases in children) last less than 6 months. Chronic forms differ in the frequency of relapses: from ITP with rare relapses to a continuously relapsing course.

Frequency of occurrence and risk factors

The frequency of ITP is, according to various sources, from 5 to 20 cases per 100,000 people. The disease occurs in both adults and children of any age, but the frequency depends on age: young adults from 20 to 40 years old are most often ill, and children and the elderly are least likely to fall ill. Girls and women suffer from ITP several times more often than boys and men. In young children, the acute form of the disease is more often observed, in adolescents and adults - chronic.

The causes and mechanism of the development of the disease are still not fully understood. But it is known that the disease can be triggered by a previous viral infection: ARVI, rubella, measles, the action of cytomegalovirus (CMV), Epstein-Barr virus (EBV), etc. In addition, pregnancy, taking a number of medications, stress, physical overload, excessive exposure to the sun, etc. ITP is not a hereditary disease.

Signs and symptoms

ITP is characterized by hemorrhagic syndrome, that is, signs of increased bleeding. As a rule, the disease occurs suddenly. Subcutaneous hemorrhages are observed: point (petechiae) and larger (ecchymosis), areas of hemorrhages can merge with each other. Bruising is easy, especially on the arms and legs. There may be hemorrhages in the mucous membranes and bleeding from them. There are frequent bleeding from the nose, gums, in girls - uterine bleeding. Less common are intestinal bleeding and the presence of blood in the urine. With a very low platelet count, deadly complications such as cerebral hemorrhages are possible. Significant blood loss with the development of severe anemia is also dangerous.

On the other hand, if the decrease in the number of platelets is not too significant, the disease may not have any obvious symptoms and be detected only by the results of a blood test, the appearance of individual bruises, and in girls - also by longer and more abundant menstruation.

Diagnostics

The first sign of ITP is a low level of blood platelets - thrombocytopenia. The rest of the blood counts remain normal. There are changes in the coagulogram, such as an increase in bleeding time. However, for an accurate diagnosis, it is necessary to exclude other causes of a decrease in platelet levels: leukemia, myelodysplastic syndrome, aplastic anemia, systemic lupus erythematosus and other autoimmune diseases, etc. When examining the bone marrow, a normal or increased number of megakaryocytes (cells from which platelets are formed) is observed ; this means that platelet deficiency is not associated with insufficient production, but with their destruction in the blood. The tests often detect antibodies to platelets in significant quantities. An enlarged spleen is uncommon for ITP.

In the diagnosis, it may be useful to study the anamnesis, including previous viral infections, as they, as mentioned above, can provoke the development of ITP.

Treatment

Therapy of patients with ITP is based on an individual approach. The need for treatment is determined by the requirement to achieve and maintain a safe level of platelets - after all, with deep thrombocytopenia, life-threatening hemorrhages are possible. Specific therapy is also determined by factors such as the degree of bleeding (severity of hemorrhagic syndrome), concomitant diseases, etc.

For example, if the level of platelets is not too low, there are no signs of hemorrhages on the skin or they gradually decrease, and there is no severe bleeding, then just careful monitoring of the patient may be recommended.

In more severe cases, however, treatment is necessary. Glucocorticoid hormones (prednisolone, methylprednisolone, dexamethasone) are often used as the first line of therapy. But their long-term use is associated with unwanted side effects. Intravenous administration of immunoglobulins ("Pentaglobin", "Oktagam", etc.) can also be used, which prevent increased destruction of blood platelets.

If the disease constantly recurs and is not amenable to therapy, then the removal of the spleen (splenectomy) may be indicated to patients. This procedure in most cases leads to success, however, it is associated with certain risks and leads to a violation of the body's immune defenses. Therefore, it is often tried to be avoided, especially in children.

Enplate (romiplostim) is a drug that has revolutionized the treatment of many patients with chronic and recurrent forms of ITP over the past 10-15 years. "Enplate" stimulates increased production of platelets, has high efficiency, speed of achieving results, good tolerability. Another drug that is effective in the chronic form of the disease is Revolade (eltrombopag). Both drugs remain effective even with long-term use, can be used on an outpatient basis (Enplate is administered subcutaneously, Revolade is used in the form of tablets), but the high cost still limits their use.

Sometimes other drugs are used, such as immunosuppressants (MabThera, Azathioprine, etc.). To improve the condition of the vessels, angioprotective drugs can be used. There are other treatment options that can be selected individually. Transfusions of donor platelets are not recommended, except in situations of life-threatening bleeding, since such transfusions increase the formation of antibodies to platelets.

Patients with ITP must comply with certain restrictions, especially during periods of exacerbations. They need to avoid any injury (which drastically limits their ability to play sports); taking aspirin and other drugs that reduce blood clotting; contact with allergens, excessive sun exposure, vaccinations, etc.

Forecast

Acute forms of ITP usually resolve within a few weeks or months, as antibodies to platelets can circulate in the blood for up to 6 months. In the chronic form of ITP, in most cases, it is possible to achieve a stable remission, but sometimes, unfortunately, the disease often takes a relapsing course. However, it is believed that on average this disease has a good prognosis, especially with the use of modern drugs.

The main symptoms of the disease are hemorrhages in the skin, mucous membranes and bleeding.

Skin hemorrhages.
- Occur spontaneously or with minor injuries, injections of drugs.
- They have various sizes - from petechial hemorrhages to extensive bruises.
- The color depends on the time of existence of the bruise. At the same time, elements of different colors (purple-bluish, yellow-green, pale yellow) can be observed, which is associated with successive stages of resorption of the bruise.
- They do not have a characteristic localization, they are located asymmetrically on any part of the skin.
- Do not rise above the surface of the skin.
- Painless.
Hemorrhages in the mucous membranes. Most often noted on the hard and soft palate, tonsils. In severe cases, hemorrhages in the eardrum, sclera (white of the eye), and vitreous body of the eye are possible.
Bleeding.
- In terms of the frequency of bleeding from the mucous membranes, the nasal mucosa occupies the first place, and gingival bleeding that occurs both spontaneously and during tooth extraction is also not uncommon.
- Less significant in terms of the incidence of bleeding from the organs of the gastrointestinal tract and kidneys.
As a rule, the examination does not find pathology of the internal organs.
Body temperature almost always remains normal.
It is important to note that enlargement of the spleen is not characteristic of thrombocytopenic purpura and distinguishes it from other diseases with a similar clinical picture.

Forms

Depending on the cause and mechanism of development, the following variants of the disease are distinguished:

actually thrombocytopenic purpura (idiopathic) - its cause is unclear;
isoimmune - develops with repeated blood transfusions (platelet mass);
transimmune thrombocytopenic purpura of newborns (neonatal) - associated with the passage of maternal blood antibodies through the placenta into the bloodstream of the child;
autoimmune thrombocytopenia - are a manifestation of other immune diseases (for example, systemic lupus erythematosus, autoimmune hemolytic anemia, etc.);
symptomatic thrombocytopenia (Werlhof symptom complex) - are non-immune in nature and occur in various diseases and conditions (B12-deficiency, hypo- and aplastic anemia (decrease in hemoglobin and red blood cells in the blood), leukemia (malignant blood disease), infectious diseases, radiation damage, taking certain medications, such as antibiotics, salicylates).

According to the flow, the forms are distinguished:

acute (up to 6 months);
chronic (more than 6 months), which in turn can occur with rare relapses (exacerbations), with frequent relapses and have a continuously relapsing course.

Three periods are distinguished during the course of the disease:

hemorrhagic crisis - manifests itself as severe bleeding and significant changes in laboratory parameters;
clinical remission - characterized by the absence of clinical manifestations with the preservation of changes in laboratory parameters;
clinical and hematological remission - implies not only the absence of bleeding, but also the normalization of laboratory parameters.

The reasons

The causes and mechanism of the development of the disease are still not fully understood.
It has been established that hereditary predisposition is important in the development of this pathology, which under the influence of various adverse factors (for example, physical and mental trauma, hypothermia, solar radiation, infections, vaccinations) can be realized in thrombocytopenic purpura.
Currently, the disease is considered as immunoallergic, in which the body produces antibodies against its own platelets (cells responsible for blood clotting), which significantly reduces their lifespan (up to several hours instead of the normal 7-10 days).

Diagnostics

Analysis of complaints of the disease:
- hemorrhages in the skin and mucous membranes that occur spontaneously or with minor injuries;
- bleeding (nasal, gingival, renal, gastrointestinal).
Analysis of the medical history:
- questioning about how the disease began and proceeded;
- lack of signs of the disease in early childhood and in blood relatives.
General inspection:
- examination of the skin, mucous membranes;
- positive endothelial tests (for example, a symptom of a tourniquet - small hemorrhages in the skin of the shoulder and forearm that occur when a tourniquet is applied to the shoulder).
Laboratory data.
- A decrease in the level of platelets (cells responsible for blood clotting) in the blood up to a single one with a normal or even elevated level of megakaryocytes (platelet progenitor cells).
- Anemia (decrease in hemoglobin and erythrocytes in the blood) in the general blood test with significant bleeding.
- Prolongation of bleeding time (up to 30 minutes or more).
- Detection of antiplatelet antibodies in the blood.
- Violation or complete absence of retraction of a blood clot (retraction - spontaneous separation of serum from a clot).
- Sternal puncture - a puncture of the sternum in order to obtain a section of the bone marrow to study its cellular composition. Detects an increase in the number of megakaryocytes, the absence or small number of free-lying platelets, the absence of other changes (for example, signs of tumor growth) in the bone marrow.
- Trepanobiopsy (examination of the bone marrow in its relation to the surrounding tissues) is performed by taking a column of bone marrow with bone and periosteum, usually from the iliac wing (the area of the human pelvis closest to the skin) using a special device - a trephine.
  - Performed if necessary to clarify the diagnosis.
  - Most accurately characterizes the state of the bone marrow.
  - With thrombocytopenic purpura, a normal ratio of fatty and hematopoietic bone marrow is detected.

Treatment of thrombocytopenic purpura

In the treatment of thrombocytopenic purpura, there is an algorithm.

If possible, before the start of drug treatment to reduce the level of antibodies and immune complexes, several sessions of plasmapheresis are prescribed (a hardware method for cleaning blood from autoantibodies and immune complexes). This allows to achieve long-term remission (a period during the course of the disease, characterized by the absence of manifestations of the disease) in almost half of the patients.
Treatment begins with the appointment of hormonal drugs (glucocorticosteroids). Therapy usually gives an effect in the first few days - first bleeding decreases, then laboratory parameters normalize.
The question of the possibility of intravenous use of normal immunoglobulin is currently being discussed, but this method is very expensive and causes only a short-term improvement.
In the absence of the effect of repeated courses of hormonal therapy, repeated heavy prolonged bleeding, leading to severe anemia (decrease in the number of erythrocytes (red blood cells) and hemoglobin (blood protein that transports oxygen)), and in the presence of profuse bleeding that threatens the patient's life, removal spleen as a source of antiplatelet antibodies. In most patients, this method of treatment allows for recovery.
In case of ineffective removal of the spleen, immunosuppressants (cytostatics) are indicated. Their use before surgical treatment is considered irrational, since the effect they exert on efficiency loses to the removal of the spleen.
With hemorrhagic syndrome, there is a need to use hemostatic (hemostatic) drugs, tamponade (tight pressing of the wound with a swab).

Complications and consequences

Complications of thrombocytopenic purpura:

hemorrhage in the brain;
profuse (massive) bleeding;
posthemorrhagic anemia (decrease in hemoglobin and red blood cells in the blood);
deaths are rare.

Prevention of thrombocytopenic purpura

Based on the fact that the causes and mechanism of development of thrombocytopenic purpura have not been studied enough, the principles of primary prevention that prevent the onset of the disease have not been developed. Secondary prevention is reduced to the prevention of recurrence of the disease:

diet with the exclusion of alcohol, spicy foods, vinegar;
prolonged exposure to the sun is not recommended;
non-steroidal anti-inflammatory drugs (aspirin, ibuprofen, indomethacin, etc.), barbiturates, caffeine and the use of physiotherapeutic methods of treatment (UVR, UHF, etc.) that can reduce the ability of platelets (cells responsible for blood clotting) to thrombosis are excluded;
prevention of infectious diseases (minimization of contact with infectious patients).

Thrombocytopenic purpura in children is one of the most common causes of increased bleeding. The disease is manifested by acute symptoms and implies certain measures of medical care for the child.

In some cases, illness disappears on its own but you shouldn't leave it unattended. Complications of pathology can negatively affect the general health of the baby and cause him significant harm.

Concept and description

Thrombocytopenic purpura in children - photo:

Thrombocytopenic purpura is a type autoimmune diseases. An ailment can occur regardless of age, but certain external and internal factors are necessary for its progression.

The disease is accompanied by increased bleeding of the child's body.

Classification

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Thrombocytopenic purpura may develop in acute and chronic form.

In the first case, complete elimination of symptoms is observed within six months. Chronic pathology occurs with regular relapses.

Additionally, the disease is classified into idiopathic and immunological type.

The causes of the first type cannot be identified, but in medical practice, special attention in this case is paid to the hereditary factor.

The immunological form is due to autoimmune abnormalities in the child's body.

Depending on the cause of the pathology, thrombocytopenic purpura is divided into the following types:

symptomatic thrombocytopenia (occurs due to the negative impact of external factors).
Isoimmune type (pathology develops against the background of blood transfusion procedures).
autoimmune thrombocytopenia (the disease is a consequence of immune diseases).
Transimmune form (diagnosed in newborns).

Symptoms and signs

Children with thrombocytopenic purpura are different weakness. They are prone to coma, their skin excessively pale. Babies with this diagnosis often feel sick. They may experience dizziness and headaches for no apparent reason.

Additionally, the pathology has symptoms associated with the appearance bleeding of the body. Signs of the disease can appear on the skin, in the general condition of the baby, or be hidden (internal bleeding).

The disease is accompanied by symptoms:

Eye hemorrhages (the shells of the eyeballs become red).
Bleeding of the skin (pinpoint bruises appear on the skin).
Excessive pallor of the skin.
Internal bleeding associated with the digestive system.
In the presence of numerous pinpoint bruises, the child does not feel discomfort.
Body temperature does not increase with pathology.
The occurrence of severe bleeding with minor injuries.
Increased bleeding of the umbilical cord.
The presence of blood impurities in the urine or feces.
Bleeding gums for no apparent reason.
Tendency to.

Complications and consequences

Fatal outcome with thrombocytopenic purpura occurs in isolated cases.

The disease is accompanied by pronounced symptoms and cannot go unnoticed.

Therapy is carried out at an early stage and reduces the risk development of relapses or complications.

If the symptoms of the pathology were ignored by adults, then the progression of a blood disorder in a child can provoke serious consequences, including the risk of death.

Complications ailments can be the following conditions:

death due to blood loss;
violation of blood flow in the brain;
posthemorrhagic;
the formation of blood clots that disrupt blood flow to internal organs;
diseases of the heart and blood vessels.

Diagnostics

Diagnosis of thrombocytopenic purpura hematologist. Many types of blood tests are required to confirm the diagnosis.

Based on the data obtained, the specialist determines the state of platelets and identifies deviations in their functioning or structure. Additionally, you may need to consult specialized doctors to compile a general clinical picture of the baby's health.

Diagnostics disease includes the following procedures:

Treatment

In the absence of symptoms of complications or the risk of their development, special treatment for thrombocytopenic purpura is not required. Doctors advise parents follow some safety rules and take care of your baby's health.

If complications appear, then the small patient is urgently hospitalized.

After discharge from the hospital can be assigned special medicines to normalize the composition of the blood and strengthen the immune system of the child.

Splenectomy

Splenectomy is mandatory procedure with thrombocytopenic purpura. Indications for its implementation are serious violations in the data of laboratory tests, the occurrence of bleeding in a child that is difficult to stop and the risk of complications.

The procedure not only restores some blood parameters, but also significantly reduces the risk of recurrence of the pathology.

Reappearance of symptoms of the disease after splenectomy considered as isolated cases..

Preparations

The need for prescribing medical treatment for the child is determined by the doctor. In some cases, special medications are not required. In the presence of complications, the treatment of the baby is carried out in a hospital setting.

Medications in this case are selected individually based on the general health of a small patient and the severity of the symptoms of the pathological process.

Types of drugs that can be assigned to a child:

corticosteroids (Prednisolone);
drugs to prevent bleeding (Adroxon, Dacyon);
vitamin C (including vitamin complexes with its content);
means of the cytostatic group (Vinblastine, Imuran);
hemostatic drugs (thrombin, epsilon-aminocaproic acid).

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Diet

When compiling a children's menu, it is important to include the maximum number of useful food.

Under the ban are lemonade, strong tea, carbonated drinks, fatty or salty foods.

The temperature regime must be controlled with special attention. Food or drinks that are too hot can cause mucous membrane burns. If a child has a tendency to pathology, then its symptoms will worsen over a short period of time.

In a child's diet must be present the following products:

red meat;
tomato and pomegranate juice;
fresh vegetables and fruits;
Fish and seafood.

In most cases, the symptoms of thrombocytopenic purpura disappear alone for six months after the first signs appear.

Parents must follow a number of important rules that will help prevent the deterioration of the child's health and reduce the risk of complications.

Particular attention should be paid prevention of any injury to the skin and mucous membranes. Otherwise, the resulting bleeding will be difficult to eliminate.

Exclusion of situations that can provoke injuries (including active sports).
Prevention of constipation with a special diet (diet must be balanced).
Particular attention should be paid to the selection of a toothbrush (the bristles should be soft and exclude gum injuries).
The child's food should not be hot (in the presence of bleeding gums or mucous membranes of the mouth, all dishes should be cooled before use).
You can not use Aspirin in the treatment of a child with such a diagnosis (the drug thins the blood).
Within five years after the treatment of pathology, the child is strictly forbidden to change climatic conditions.

Thrombocytopenic purpura in children - federal.

Forecast

Thrombocytopenic purpura has a favorable prognosis only in the presence of timely treatment of the disease and constant monitoring of health status child from his parents. Pathology is characterized by a long remission.

For the treatment of nervous tics in children, you will find on our website.

Prevention

Special primary prevention of thrombocytopenic purpura in medical practice not developed.

To prevent the disease, it is necessary to follow the elementary rules aimed at controlling the nutrition of the baby and attentive attitude to the state of his health.

Secondary prevention includes specific recommendations that may exacerbate the pathology. Children diagnosed with thrombocytopenic purpura are forbidden to engage in active sports and must be regularly examined by a hematologist.

Prevention measures disease are the following recommendations:

Exclusion of stressful situations, physical and psychological overstrain of the child.
Compliance with the rules of a balanced diet with temperature control of food.
Replenishment of vitamin reserves with special preparations designed for children.
The child's diet should not contain foods containing vinegar.
All diseases, regardless of etiology, must be treated fully and in a timely manner.
Reception of antipyretics and potent medicines must be agreed with the doctor.

In some cases, it is not possible to avoid the development of thrombocytopenic purpura in children, especially in the presence of a hereditary factor.

If the child has skin bruises or has tendency to difficult to control bleeding, it is necessary to consult with a hematologist as soon as possible. Timely diagnosis of pathology will help to avoid complications and the development of a chronic form of the disease.

You can learn about thrombocytopenic purpura in children from this video:

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Hematologist

Higher education:

Hematologist

Samara State Medical University (SamSMU, KMI)

Level of education - Specialist
1993-1999

Additional education:

"Hematology"

Russian Medical Academy of Postgraduate Education

Thrombocytopenic purpura refers to blood diseases associated with a lack of platelets. It belongs to the group of hemorrhagic diathesis.

What is thrombocytopenic purpura?

A decrease in the number of platelets leads to a sharp drop in blood clotting. The vessels suffer from a lack of nutrition, their inner layer is damaged, and the permeability of the walls for red blood cells increases. The process is accompanied by a failure of the immune system, which begins to fight with the cells of its own body - platelets.

Spontaneous bruising occurs on the body in the form of small red spots or bruises. They may appear as pinpoint hemorrhages (petechiae), coalesce, and form streaks or large spots. From the color of these spots, the disease got its name - purpura. In fact, the color of hemorrhages can vary from purple-red to yellow-green.

Bleeding appears not only in the form of skin rashes. Small hemorrhages occur on the mucous membranes in the nose, in the oral cavity, on the sclera of the eyes, in adipose tissue, on the internal organs and on the brain membrane. Most often, the disease manifests itself in childhood. Thrombocytopenic purpura in adults occurs just as often and mainly in women (their incidence is 3 times higher than in men).

Classification of thrombocytopenic purpura

The classification of purpura is carried out according to the form of the course of the disease and according to the mechanism of occurrence. According to the flow, they distinguish:

acute;
chronic;
cyclically relapsing.

Acute thrombocytopenia often occurs in childhood and lasts for six months. During this time, the level of platelets in the blood is restored to normal. It does not recur in the future. Adults suffer from the chronic form, its duration is more than 6 months. The recurrent form is manifested by a change in cycles of normalization and a drop in platelet levels.

Classification according to the mechanism of occurrence

According to the mechanism of occurrence, thrombocytopenic purpura is classified as follows:

idiopathic thrombocytopenic purpura or Werlhof's disease;
thrombotic thrombocytopenic purpura;
autoimmune thrombocytopenic purpura.

The etiology of the disease is different. It can occur for no apparent reason. Such purpura is called idiopathic and is not associated with other pathologies. For the first time this condition was described by the doctor Paul Werlhof back in the 18th century, therefore the second name of idiopathic thrombocytopenic purpura is Werlhof's disease.

Thrombotic purpura

Thrombotic thrombocytopenic purpura is a type of disease that should be considered separately. It is severe and has a poor prognosis. Most often people from 30 to 40 years old get sick. It is also characterized by a decrease in platelets in the blood and the appearance of red spots on the skin.

However, its course is fundamentally different from thrombocytopenic purpura. Damaged platelets stick together and clog arterial capillaries, which leads to organ ischemia. The brain and tissues of the lungs, heart and kidneys suffer. Thrombotic thrombocytopenic purpura initially takes an acute form and develops quite rapidly, accompanied by bleeding, fever, neurological symptoms - disorientation, tremor, visual impairment (double vision), convulsions, and other severe manifestations that can lead to coma. Always accompanied by hemolytic anemia. Often the disease ends with the death of the patient from renal failure.

Immune forms of thrombocytopenia

One of the causes of damage and death of platelets is the attack of their own immune cells. These attacks can be of different nature:

The transimmune form of the disease occurs in utero in the fetus, to which antiplatelet antibodies are transmitted from the mother. In the first month of life, the child has a reduced number of platelets in the blood.
Isoimmune develops when someone else's blood is transfused.
The heteroimmune form is characterized by a change in the antigenic structure of platelets. At the same time, the cells of the immune system stop recognizing them and attack them as "strangers". Doctors believe that these changes are due to past viral diseases or under the influence of certain drugs. If the heteroimmune form of thrombocytopenia lasts more than 6 months, it is transferred to the category of autoimmune.
autoimmune form. It also includes idiopathic thrombocytopenic purpura in adults, when the cause of the attack of one's own immunity on platelets is unknown.

The occurrence of immune thrombocytopenic purpura is possible at any age. It often becomes recurrent or chronic. Women are more susceptible to it.

Causes of the disease

The exact causes of thrombocytopenic purpura are still a matter of controversy among physicians. In almost 50% of cases, it is diagnosed as idiopathic. In isolated cases, it is hereditary and is the result of a violation of the production of platelets by the bone marrow. The most likely factors provoking the disease are:

viral diseases;
the effect of radiation;
metastases and bone marrow tumors;
mechanical damage to platelets during vascular prosthesis surgery;
reaction to vaccination and administration of gamma globulin;
the introduction of cytostatics during chemotherapy;
some oral contraceptives.

In rare cases, prolonged blood stasis and pregnancy can be the cause. Perhaps the appearance of secondary purpura, as a concomitant pathology in other diseases:

connective tissue lesions (systemic diseases);
leukemia;
hemophilia;
oncology with metastases in the bone marrow;
thrombocytopathies and some others.

Symptoms

Usually the disease develops rapidly. This is due to the catastrophic shortening of the life of platelets. Its normal duration is 7-10 days. And under the influence of damaging factors, it is reduced to several hours. Symptoms are approximately the same for all forms of thrombocytopenic purpura, differ only in the intensity of manifestation:

the appearance of a chiseled red rash or large subcutaneous hematomas;
hemorrhages when the skin is injured (for example, after an injection);
obvious bleeding from the mucous membranes;
hidden bleeding from the eardrum, stomach and intestinal bleeding (stool stained with blood);
anemia that develops against the background of constant blood loss;
nocturnal hemorrhages.

Possible cough with blood, hemorrhage in the brain and vitreous body of the eye. The body is covered with a rash only in front. Its asymmetric localization is characteristic. The rash may be dry or bleeding. Autoimmune thrombocytopenia can cause an enlarged spleen. The rash is sometimes accompanied by fever. Boys often have nosebleeds, girls have uterine bleeding.

Usually the course of the disease goes through three stages:

hemorrhagic crisis, when the platelet count drops to a critical level;
clinical remission, in which external manifestations disappear, but a shift in laboratory tests remains;
Clinical and hematological remission, when external symptoms disappear and blood counts return to normal.

Diagnostics

The primary diagnosis of thrombocytopenic purpura is a visual therapeutic examination of external manifestations. Bleeding is also taken into account. Laboratory diagnostics includes a complete blood count for platelets, a bone marrow myelogram, and a biochemical blood test. In children, treatment is prescribed after examination and identification of symptoms. Differential diagnosis of secondary purpura is carried out.

Thrombotic purpura is diagnosed additionally in terms of the number of leukocytes, recticulocytes and the state of erythrocytes (they are characterized by an altered form). Indicators in the biochemical blood test increase (bilirubin, creatinine, urea, fibrinogen breakdown products appear).

Treatment

In the presence of hemorrhagic symptoms, treatment of thrombocytopenic purpura is carried out only in a hospital. With this disease, strict bed rest is indicated. First of all, the patient needs to stop bleeding and conduct therapy aimed at increasing the level of platelets. The first drugs used for treatment are hemostatic (Ascorutin, Thrombin). Then hormone therapy (Prednisolone) and immunoglobulins are prescribed. Reception of corticosteroid drugs continues for at least three months.

Transfusion of someone else's thrombus mass is almost never used in treatment, since it may not take root. With strong signs of anemia, washed red blood cells can be transfused - blood devoid of white blood cells and platelets. If the above methods do not work, they resort to a radical method - removal of the spleen. The operation is done only with a high risk of its rupture and incessant bleeding.

After the treatment, the patient is discharged and becomes a dispensary record in the clinic. Parents are responsible for children. They are obliged to provide the child with a normal diet, periodic medical examination and minimize the factors that provoke the recurrence of the disease. It is necessary to exclude any allergenic products and protect the child from injury.

After treatment, adults are categorically contraindicated in taking blood-thinning drugs (for example, all types of aspirin), hypnotics and sedatives, and caffeine. Dispensary registration for the disease lasts at least two years.