Hemorrhagic rash (purpura on the skin) in children and adults: causes, what diseases it is a symptom of, treatment. Hemorrhagic purpura treatment

About 40% of all hemorrhagic rashes are associated with thrombocytopenic purpura. Its prevalence ranges from 1 to 13 people per 100 thousand population, depending on the region.

Among all hemorrhagic diathesis, thrombocytopenic purpura ranks first in prevalence among preschool children; in adults, the syndrome is less common and affects mainly women.

What is thrombocytopenic purpura?

Thrombocytopenic purpura (Werlhof's disease, ITP, primary immune thrombocytopenia) is a disease characterized by a reduced level of platelets in the blood ().

The lifespan of platelets ranges from 7 to 10 days.

With thrombocytopenic purpura, the immune system suppresses its own blood cells, and they die within a few hours. The consequence of this is increased bleeding time.

Thrombocytopenic purpura is divided into acute, recurrent and chronic forms of the disease.

1. Mostly children suffer from the acute form, the disease lasts about 6 months, after recovery the level of platelets in the blood normalizes, there are no relapses.
2. The chronic form lasts more than 6 months and affects adults.
3. The recurrent form has a cyclical course: relapses are followed by periods of relief. Based on the frequency of exacerbations, the disease is divided into frequent, rare, and continuously recurrent.

Depending on the cause of the disease, thrombocytopenic purpura is divided into forms:

• isoimmune (alloimmune) thrombocytopenia occurs during fetal development and is associated with:
  • penetration of antiplatelet antibodies through the placenta from mother to child, a reduced level of platelets is observed in the prenatal period and in the first months after birth;
  • antiplatelet antibodies appear after blood transfusion;
• heteroimmune (hapten) thrombocytopenia occurs as a result of changes in platelet antigens; as a result, the immune system perceives its own blood cells as foreign. This reaction sometimes occurs as a result of a viral or bacterial infection, or after taking certain medications. If heteroimmune thrombocytopenia does not disappear within six months, then it is considered autoimmune.
• Autoimmune thrombocytopenia (AITP) has many forms, including idiopathic thrombocytopenic purpura (Werlhof's disease), the cause of which is unknown.

Causes

The reasons for the development of thrombocytic purpura still remain not fully established.

• 45% of cases of thrombocytic purpura are idiopathic (Werlhof's disease), that is, the causes have not been established.
• 40% of cases of this pathology are associated with a previous infectious disease and occur 2-3 weeks after recovery. Most often, an infection of viral origin is important (chickenpox, HIV infection, whooping cough, etc.), much less often - bacterial (malaria, typhoid fever, etc.)

Thrombocytopenic purpura can also occur due to:

• severe hypothermia;
• injuries;
• excessive insolation (ultraviolet radiation);
• radiation;
• complication after vaccine administration;
• taking certain medications (barbiturates, salicylic acid, antibiotics, estrogens, arsenic, mercury salts);
• disruption of the hematopoietic system;
• neoplasms localized in the bone marrow;
• vascular prosthetics due to mechanical damage to blood cells;
• taking certain oral contraceptives.

Risk factors

Thrombocytopenic purpura can occur in children and adults of any age, but there are factors that increase the risk of developing this condition:

• being female (thrombocytopenic purpura occurs three times more often in women than in men);
• a recent infectious disease increases the risk of developing the disease, especially in children;
• heredity (the disease in close relatives increases the risk of thrombocytopenia);
• frequent stress.

Symptoms of thrombocytopenic purpura

In Werlhof's disease, the main symptoms are hemorrhages.

Photo of idiopathic thrombocytopenic purpura

Werlhof's disease has three stages in its course:

• hemorrhagic crisis– the manifestations are pronounced; a blood test shows a reduced level of platelets;
• clinical remission– external signs of thrombocytopenia fade away, but its characteristic changes in blood tests are still present;
• clinical and hematological remission– the tests come back to normal, there are also no clinical signs of the disease.

The first signs of thrombocytopenia appear when the level of platelets in the blood decreases below 50*109/l, this occurs approximately 2-3 weeks after the factor that caused the disease was exposed.

Acute thrombocytopenic purpura

The disease begins suddenly: hemorrhages appear on the skin and mucous membranes (see photo above), bleeding begins, the general condition worsens, the skin becomes pale, and blood pressure drops.

Body temperature can reach 38 degrees. The lymph nodes become inflamed and painful.

Chronic thrombocytopenic purpura

Petechiae on the lateral surface of the tongue.

Signs of chronic disease

The main symptom of the disease is rash. They appear on the skin and mucous membranes, are painful, and vary in size.

Rashes can be of several types:

• petechiae (small dots);
• vibex (rash collects in groups or stripes);
• large spots, including petechiae and stripes.

The color of fresh rashes is purple. Faded rashes are yellow or green in color. The rash on the skin can be wet or dry.

In case of wet rashes, bleeding may occur, especially at night.

Common location: on the chest, abdomen, upper and lower extremities, rarely on the face and neck. At the same time, a rash appears on the mucous membranes.

Some locations are inaccessible for inspection without special equipment: the eardrum, serous membrane of the brain and other organs.

An important symptom of the disease is bleeding of varying intensity. The most commonly observed bleeding:

• nose bleed;
• from the gums and in places of extracted teeth;
• after surgery to remove tonsils;
• when coughing from the respiratory tract;
• from the digestive tract with vomit or urine;

Bleeding occurs either simultaneously with skin rashes or after.

The body temperature in chronic thrombocytopenic purpura is not elevated, sometimes there is a rapid heartbeat, and in children the lymph nodes become enlarged and painful.

Thrombotic form of idiopathic thrombocytopenic purpura.

This form of the disease is the most dangerous of all.

Characterized by an acute, spontaneous onset and malignant course.

Due to the formation of hyaline blood clots, the blood supply to various organs is disrupted.

Idiopathic thrombocytopenic purpura (ITP) of the thrombotic type is characterized by the following symptoms:

• hemorrhagic rash;
• fever;
• (it is this that causes death);
• convulsions, sensitivity disorders;
• joint pain;
• neurosis, confusion, coma;
• abdominal pain.

Diagnostics

To make a diagnosis of Werlhof's disease, a patient is interviewed and examined. Laboratory methods for examining blood, urine and the spinal cord are used to confirm the presence of the disease.

In chronic cases, hematological parameters may be within normal limits.

During the interview, a hematologist (a doctor specializing in blood diseases) finds out the presence of factors in the medical history that contribute to the development of thrombocytopenic purpura: viral infection, medication, vaccination, exposure to radiation, etc.

Examination of the patient reveals a characteristic sign of this disease - a hemorrhagic rash on the skin and mucous membranes. The doctor can also conduct a number of tests that make it possible to detect hemorrhages in the skin:

• Cuff test used in adults and children over 3 years of age. A blood pressure cuff is placed on the patient's arm; if pinpoint hemorrhages are detected after 10 minutes, the test is considered positive.
• Tourniquet test used only in adults. When a medical tourniquet is applied, hemorrhages appear at the point of pressure.
• "Pinch method"- after a small pinch, a bruise forms.

A general blood test reveals deviations in the level of hemoglobin (noted with significant blood loss) and platelets. shows the rate of blood clotting, the presence of antiplatelet antibodies, reduced or absent clot retraction. Red blood cells are detected.

In severe cases, a red bone marrow biopsy is performed. When examining a biopsy specimen, a normal or increased content of megakaryocytes and the presence of their immature forms are revealed.

In the case of characteristic clinical symptoms, treatment can be started immediately, without waiting for the results of laboratory tests.

Treatment of Werlhof's disease

If thrombocytopenic purpura does not cause complications, there is no significant bleeding, platelet counts in the blood are not lower than 50 * 109 / l, medical tactics consist of observation - no treatment is required.

When the platelet level decreases to 30-50*109/l, treatment is necessary for patients at risk for bleeding (arterial hypertension).

If the platelet level drops below 30*109/l, urgent hospitalization is required.

Conservative therapy

Conservative therapy includes the use of medications that suppress autoimmune processes and reduce vascular permeability:

• glucocorticosteroids (Prednisolone) have a treatment effect within 1-2 weeks;
• globulins (Immunoglobulin G), increases platelet levels;
• interferons (Interferon A2) are used when glucocorticosteroids are powerless;
• cytostatics (Cyclophosphamide, Imuran, Vinblastine, Vincristine and Azathioprine);
• vitamins PP and C, calcium salts, aminocaproic acid.

To stop external bleeding, a hemostatic sponge is used; in case of internal bleeding, drugs are administered to stop the bleeding.

In severe cases, plasmapheresis is used - transfusion of blood components and platelets.

Surgery

In some cases, idiopathic thrombocytopenic purpura does not respond to conservative therapy, then removal surgery is performed - splenectomy.

Significant improvement is immediately observed, but there is a risk of postoperative complications and the body's resistance to infectious diseases is significantly reduced.

Splenectomy is performed for a number of indications:

• the duration of the disease is more than 1 year, 2-3 periods of exacerbation, ineffectiveness of glucocorticosteroid therapy;
• contraindications to taking adrenocorticosteroids;
• relapse of thrombocytopenia after a course of drug therapy;
• severe course of idiopathic thrombocytopenic purpura, hemorrhages in vital organs.

Traditional medicine

To treat thrombocytic purpura, in addition to medications, medicinal plants with hemostatic properties are used. Among them:

• stinging nettle;
• horsetail;
• burnet;
• shepherd's purse;
• viburnum bark;
• sorrel;
• lemon balm;
• shepherd's purse;
• ginseng;
• Potentilla gossamer.

Diet

It is important that the food consumed is slightly warm or cool. Fresh vegetables and fruits are useful, but you need to make sure that they do not cause an allergic reaction.

Prohibited:

• salty foods;
• smoked;
• spices;
• fast food products;
• drinks: carbonated, alcoholic, coffee.

Forecast

In adults, thrombocytopenic purpura ends in complete recovery in 75% of cases, in children - in 90% of cases. The occurrence of serious complications is possible only in the acute period of the disease.

The likelihood of death with the thrombotic variant of thrombocytopenic purpura depends on the extent of the lesion and the degree of damage to the brain, cardiovascular system, kidneys and other organs.

Patients with a history of this pathology need constant medical supervision, exclusion of medications that negatively affect blood clotting, and a review of lifestyle and nutrition.

Prevention

Preventive measures for thrombocytopenic purpura are aimed at preventing exacerbations. They involve maintaining platelet and hemoglobin levels in the blood and include:

• exclusion of allergens in food (spicy, fried, alcohol);
• refusal of medications that affect platelet aggregation (ibuprofen, caffeinated drugs, sleeping pills, aspirin);
• abstaining from prolonged exposure to the sun;
• limit vaccination, refuse anti-influenza vaccine;
• avoiding contact with infectious patients;
• avoidance of traumatic sports;
• exclusion of strict diets;
• minimizing stressful situations.

After discharge from the hospital, the patient is registered at the dispensary at his place of residence - he is observed by a doctor for at least 2 years.

However, a large share of responsibility for health falls on the shoulders of the patient or his parents in the event of a child’s illness.

Given that thrombocytopenic purpura is common in children, it is very important to educate the entire family about how to prevent this disease.

Videos on the topic

In adults, this is a disorder that can cause large bruises and bleeding to occur quickly. Typically, blood loss occurs due to an extreme drop in the level of platelets - cells that help blood clot.

In some sources, the pathology in question is also called immune thrombocytopenia. It affects both adults and children, but in young patients this condition usually develops against the background of a viral infection and goes away on its own, without requiring special treatment. In adults, the disease in most cases is protracted.

If you have no signs of bleeding, and your platelet count, although low, is generally satisfactory, your doctor will most likely not prescribe treatment. In rare cases, however, the platelet count drops to a critical minimum, causing life-threatening internal hemorrhages. To treat such complications, immediate medical intervention is required.

Symptoms

In adults (photo above) it is not always accompanied by external manifestations of pathology. If characteristic symptoms develop, the following signs of the disease can be noted:

• excessively rapid formation of large bruises (hematomas);
• superficial bleeding into the skin, which looks like a rash of tiny reddish-purple spots (petechiae) and is most often localized on the legs;
• bleeding from the gums or nose;
• presence of blood in urine or stool;
• unusually heavy menstrual bleeding.

When to see a doctor

Thrombocytopenic purpura in adults can be a serious concern, in which case it is best to make an appointment with a qualified specialist in advance.

If you are bleeding, call an ambulance. The bleeding associated with this disease cannot be controlled using conventional home remedies or typical methods of stopping the flow of blood. So, for example, physical pressure on the corresponding part of the body will not help you. Experts recommend seeking medical help as soon as possible.

Causes

Purpura in adults sometimes develops because the body's immune system mistakenly attacks and destroys platelets. The term "idiopathic" means "developed from an unknown cause"; thrombocytopenia is so characterized in cases where doctors are unable to determine what exactly provoked an inadequate reaction of the immune system.

In children, it is usually easier to establish the prerequisites for the pathology, since this disorder most often follows an infectious disease (mumps or influenza). It is assumed that it is the infection that disrupts the normal functioning of the immune system.

Increased platelet breakdown

If the initial diagnosis is thrombocytopenic purpura in adults, the clinical manifestations are usually associated with an immune disorder. Antibodies attach to platelets, marking them as cells to be destroyed. The spleen, designed to fight external infections, recognizes antibodies and removes platelets from the body. As a result of this mistaken identity, there are far fewer platelets circulating in the blood than normal.

The normal platelet count ranges from 150 to 450 thousand cells per microliter of circulating blood. In people diagnosed with thrombocytopenic purpura (adults), tests reveal only 20 thousand of the required cells. Since platelets help blood clot, a decrease in their number leads to an increased risk of bleeding and internal hemorrhage. A level of 10 thousand cells is life-threatening. With such a significant decrease in the number of platelets, internal hemorrhage can begin even without any trauma.

Risk factors

Thrombocytopenic purpura in adults (the photo used at the beginning of the article demonstrates the external manifestations of pathology) is detected at any age, but scientists have identified two signs of the disease that increase the likelihood of development. This:

• Gender. Women are diagnosed with this disorder on average two to three times more often than men.
• A recent viral infectious disease. Mumps, measles and respiratory infections can cause the characteristic symptoms of thrombocytopenia, but the association with viruses is more common in children.

You should beware of the risk of complications. The most serious of them is one that can be fatal.

Pregnancy

Although thrombocytopenic purpura in adults is diagnosed even in pregnant women, in most cases this maternal pathology does not affect the health of the newborn child. Experts, however, recommend checking the level of platelets in the baby’s blood immediately after birth.

If you are pregnant and test results indicate a decrease in platelet levels, you are at risk, since the likelihood of severe postpartum bleeding is very high. In such cases, the observing gynecologist or maternity hospital doctors should discuss with you the available treatment methods that are safe for the child.

Diagnostics

In order for a doctor to make an accurate diagnosis of “thrombocytopenic purpura” (in adults), the diagnosis must be carried out according to the differential principle. You should immediately rule out other possible causes of bleeding and low platelet counts, such as chronic diseases or side effects of medications you are taking.

The specialist will ask you questions about your medical history, perform an initial medical examination and order one or more standard diagnostic tests. The latter include:

• General blood analysis. Based on a blood sample taken from the patient, the total ratio of blood cells, as well as the specific number of platelets, is determined. With thrombocytopenia, the latter parameter will be abnormally low, although the level of white and red blood cells will remain average.
• Clinical blood test. This test is often done to confirm platelet levels obtained through a complete blood count. A sample taken from the patient is placed on a special glass and examined under a microscope.
• This research method is recommended only for adult patients with a preliminary diagnosis of thrombocytopenic purpura (in adults, the reaction to such an event is more adequate, they tolerate the procedure more easily).

Platelets are produced inside the bone marrow, the soft, spongy tissue located in the center of large bones. In some cases, the doctor may remove a sample of the bone tissue and the bone marrow inside it using a biopsy. Some specialists prefer to take samples for analysis using a procedure called aspiration, in which only part of the liquid component is removed from the bone marrow. Most often, both procedures are performed simultaneously and are collectively called a bone marrow test.

In idiopathic thrombocytopenic purpura, the bone marrow is free of abnormalities because the decrease in platelet levels is caused by the destruction of cells in the bloodstream and dysfunction of the spleen, and not by problems with platelet production.

Treatment

Mild thrombocytopenic purpura in adults, the causes of which have not been reliably established, usually does not require specific treatment - it is enough to see a doctor regularly and check platelet levels from time to time. Children almost never require therapy and the disorder goes away on its own. In adults, however, effective treatment may be needed if the condition is severe or prolonged (chronic).

Treatment options vary, ranging from drugs to increase platelet counts to surgery to remove the spleen (splenectomy). It is advisable to discuss treatment options with your doctor in advance, along with the pros and cons of surgery. Some patients believe that the side effects of taking medications cause them more inconvenience and discomfort than the actual consequences of the disease.

Medications

Your doctor will discuss with you the side effects and dosage of the medications and dietary supplements you are currently taking. It is possible that some of them contribute to a decrease in the level of platelets in the circulating blood. Aspirin, Ibuprofen, Ginkgo biloba and Warfarin have such side effects.

Main list

When a diagnosis of thrombocytopenic purpura is confirmed in adults, treatment may consist of the following medications:

1. Medicines that suppress the immune system. Many patients are prescribed oral corticosteroids such as Prednisone at the beginning of therapy. By reducing the activity of the immune system, this drug helps increase platelet counts. Once the overall level reaches an acceptable level, you can gradually stop taking the medicine under the supervision of a doctor. The course of treatment takes from two to six weeks.

The problem is that thrombocytopenic purpura in adults, the symptoms of which have disappeared after taking Prednisone, may return again after the end of therapy. Doctors usually prescribe a new course of corticosteroids, but this treatment cannot be continued indefinitely due to the serious side effects associated with this type of medication. Overdose may result in cataracts, increased blood sugar levels, increased risk of infection, and osteoporosis (weakening bones).

2. Injections to increase the total number of blood cells. If corticosteroids don't help, doctors prescribe an injection of immune globulin. This drug is also used in cases where the patient has severe bleeding that requires immediate surgical intervention. The effect wears off within a couple of weeks. Possible side effects include headaches, vomiting and low blood pressure.

3. Drugs that accelerate platelet production. Medicines such as Romiplostim and Eltrombopag (Promacta) help the bone marrow produce more platelets. Side effects may include headaches, dizziness, nausea and vomiting.

4. Other immunosuppressants. "Rituximab" ("Rituxan") helps reduce the intensity of the abnormal reaction of the immune system, as a result of which normal platelets are destroyed. Potential side effects include skin rashes, low blood pressure, fever and sore throat.

If medications don't work

If your thrombocytopenic purpura progresses (in adults, eating a healthy lifestyle, avoiding alcohol and taking medications sometimes does not help get rid of the problem), your doctor may recommend surgery. quickly eliminates pathology, as the surgeon cuts out the main platelet destroyer. It must be taken into account, however, that you will be at risk of postoperative complications. In addition, people without a spleen are constantly at increased risk of contracting infectious diseases.

Alternative Methods

If, for health reasons or other reasons, you cannot afford surgery, the specialist may prescribe new medications - for example, Azathioprine (Imuran, Azasan). However, more powerful drugs have more serious side effects. Azathioprine, in particular, causes fever, headaches, nausea, vomiting and muscle pain.

For chronic disorders

Have you been diagnosed with thrombocytopenic purpura? In adults, the occurrence of this pathology requires changes in lifestyle. Since the disease is accompanied by an increased risk of bleeding, you should avoid potentially dangerous contact sports (boxing, martial arts, football) and treat your own body as carefully as possible.

Typically, thrombocytopenic purpura first develops in children aged 2-6 years (up to 10 years), regardless of gender. In adults, the disease is not as common, and women are more likely to suffer from it.

Characteristic of this disease is a decrease in the number of platelets in the blood serum below the level of 100 x10 9 /l against the background of sufficient formation in the bone marrow, and the presence of antibodies on the surface of platelets and in the blood that cause their destruction.

Depending on the duration and cyclicality of the course of the disease, there are several forms of thrombocytopenic purpura:
1. Spicy.
2. Chronic.
3. Recurrent.

The acute form is characterized by an increase in the level of blood platelets of more than 150x10 9 /l within 6 months from the date of development of the disease, in the absence of relapses (repeated cases of the disease) subsequently. If recovery of platelet levels is delayed for more than 6 months, a diagnosis of chronic thrombocytopenic purpura is made. When their number decreases below normal again after their recovery, recurrent thrombocytopenic purpura occurs.

Causes of thrombocytopenic purpura

• Rifampicin;
• Vancomycin;
• Bactrim;
• Carbamazepine;
• Diazepam;
• Sodium valproate;
• Methyldopa;
• Spironolactone;
• Levamisole;

Symptoms

Hemorrhages can occur not only on the mucous membranes of the oral cavity and tonsils, but also in the eardrum, vitreous body, sclera and fundus of the eye. Rarely, cerebral hemorrhage is possible, which significantly worsens the patient’s condition. This is preceded by the appearance of dizziness and headache, as well as bleeding in other organs.

When the platelet level decreases to less than 50x10 9 /l, nosebleeds and gum bleeding appear, which are more dangerous when a tooth is removed. In this case, bleeding occurs immediately and usually does not resume after it stops. In teenage girls with thrombocytopenic purpura, uterine bleeding during menstruation poses a certain danger.

Stages of thrombocytopenic purpura

Diagnostics

The examination complex includes the following diagnostic procedures:

• complete blood count with platelet count;
• 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 to the Epstein-Barr virus, parvovirus in the blood.

Thrombocytopenic purpura in children

In children under 2 years of age, the infantile form of thrombocytopenic purpura is registered. In this case, the disease begins acutely, without the presence of a previous infection, and is extremely difficult: the platelet level drops below 20x10 9 /l, treatment is ineffective, and the risk of chronicity of the disease is very high.

The clinical manifestations of ITP depend on platelet levels. The onset of the disease is characterized by the appearance of spotty-bruised rashes on the skin and mild hemorrhages on the mucous membranes. When the platelet level decreases to less than 50 x10 9 /l, various bleedings may occur (nasal, gastrointestinal, uterine, renal). But most often, large “bruises” in places of contusions attract attention; there may be hematomas during intramuscular injections (injections). An enlarged spleen is characteristic. A general blood test records thrombocytopenia (decreased platelets), eosinophilia (increased number of eosinophils), anemia (decreased hemoglobin).

Treatment

Treatment of idiopathic thrombocytopenic purpura is aimed at reducing the production of antiplatelet antibodies and preventing their binding to platelets.

Diet for thrombocytopenic purpura

Drug therapy

• The general dose is prednisolone at a dose of 1-2 mg/kg per day for 21 days, then the dose is gradually reduced until complete withdrawal. A repeat 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 10-30 mg/kg per day, with rapid 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).

With long-term use and individually, each patient may experience side effects from taking glucocorticoids: an increase in blood glucose levels and a decrease in potassium levels, stomach ulcers, decreased immunity, increased blood pressure, growth retardation.

2. Immunoglobulins for intravenous administration:

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

With the use of immunoglobulins, headaches, allergic reactions, increased body temperature to high levels and chills may occur. To reduce the severity of undesirable effects, Paracetamol and Diphenhydramine are prescribed orally, and Dexamethasone intravenously.

3. Interferon alpha.
Indicated for chronic purpura in case of ineffective treatment with glucocorticoids. 2x106 units of interferon-alpha are injected under the skin or into the muscle for a month, 3 times a week, every other day.

Often during treatment with interferon appear

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 has also been proposed to use the term “primary immune thrombocytopenia”.

Antibodies to his own platelets are formed in the patient’s body, the rate of destruction of blood platelets increases several times, and a deficiency of these cells occurs. 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 (which make up the majority of cases of the disease 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.

Incidence 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 affected, and children and the elderly are least likely to get sick. Girls and women suffer from ITP several times more often than boys and men. In young children, an acute form of the disease is more often observed, in adolescents and adults - a chronic form.

The causes and mechanism of development of the disease are still not fully understood. But it is known that the disease can be triggered by a previous viral infection: acute respiratory viral infection, rubella, measles, cytomegalovirus (CMV), Epstein-Barr virus (EBV), etc. In addition, possible provoking factors include pregnancy, taking a number of medications, stress, physical overload, excessive sun exposure, 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: pinpoint (petechiae) and larger (ecchymosis), areas of hemorrhage can merge with each other. Bruises easily, especially on the arms and legs. Hemorrhages into the mucous membranes and bleeding from them may occur. Bleeding from the nose, gums, and uterine bleeding in girls are common. Intestinal bleeding and the presence of blood in the urine are less common. If platelet levels are very low, life-threatening 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 platelet count is not too significant, the disease may not have any obvious symptoms and can be detected only by the results of a blood test, the appearance of isolated bruises, and in girls, also by longer and heavier menstruation.

Diagnostics

The first sign of ITP is a low level of blood platelets - thrombocytopenia. Other blood counts remain normal. Changes in the coagulogram are observed, such as an increase in bleeding time. However, for an accurate diagnosis, it is necessary to exclude other causes of low 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 due to insufficient production, but to their destruction in the blood. Tests often detect antibodies to platelets in significant quantities. An enlarged spleen is not typical for ITP.

When diagnosing, it may be useful to study the medical history, including previous viral infections, since, as mentioned above, they can provoke the development of ITP.

Treatment

Therapy for 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 platelet level is not too low, there are no signs of hemorrhages on the skin or they are gradually decreasing, and there are no severe bleedings, then simply 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, Octagam, etc.) can also be used, which prevent increased destruction of blood platelets.

If the disease constantly recurs and does not respond to therapy, then patients may be advised to remove the spleen (splenectomy). This procedure is successful in most cases, but is associated with certain risks and leads to disruption of the body's immune defense. Therefore, they often try to avoid it, especially in children.

"Enplate" (romiplostim) is a drug that in the last 10-15 years has revolutionized the treatment of many patients with chronic and recurrent forms of ITP. “Enplate” stimulates increased platelet production, is highly effective, quickly achieves results, and is well tolerated. Another drug effective for the chronic form of the disease is Revolade (eltrombopag). Both drugs remain effective even with long-term use and can be used on an outpatient basis (“Enplate” is administered subcutaneously, “Revolade” is used in tablet form), but the high cost still limits their use.

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

Patients with ITP must observe certain restrictions, especially during periods of exacerbation. They need to avoid any injuries (which severely 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 platelet antibodies can circulate in the blood for up to 6 months. In the chronic form of ITP, in most cases it is possible to achieve stable remission, but sometimes, unfortunately, the disease takes a frequently relapsing course. However, it is believed that on average this disease has a good prognosis, especially with the use of modern medications.

Hemorrhagic vasculitis is a disease accompanied by inflammatory processes of microvessels and occurs with thrombus formation. Refers to systemic vasculitis. It affects internal organs and blood vessels - arterioles, venules and capillaries. Most often, the intestines and kidneys suffer from pathology.

According to ICD-10, this is allergic purpura. It mainly develops in children aged 5-14 years, occurring in 23-25 ​​people out of 10 thousand. Isolated cases of the disease affecting children under 3 years of age have been recorded.

Causes

When affected by vasculitis, immune antibodies appear in the blood, which during circulation are deposited on the walls of blood vessels and cause local damage. Aseptic inflammatory processes form in the affected areas, which increases vascular permeability. Fibrin is deposited in the lumen of the vessels, microthrombosis develops, and hemorrhagic syndrome appears.

Hemorrhagic vasculitis in adults, in contrast to the course of the disease in children, is characterized by mild symptoms. The abdominal form occurs in half of the cases.

Classification

Henoch-Schönlein purpura is divided into types:

• skin-articular (simple, necrotic and with edema);
• abdominal;
• renal;
• mixed.

Hemorrhagic purpura is classified according to the duration of the disease:

• fulminant (more often in children under 5 years of age);
• acute (passes within 1 month);
• subacute (recovery occurs within 3 months);
• protracted (lasts up to 6 months)
• and chronic.

Hemorrhagic vasculitis is divided into 3 degrees of activity:

• I degree - increase in ESR to 20 mm/hour. The general condition is satisfactory, the body temperature is low or within normal limits, there may be small rashes on the skin.
• II degree - ESR 20-40 mm/hour, dysproteinemia. There is an increased content of neutrophils, leukocytes and eosinophils in the blood. The severity of the course is average, manifestations on the skin are very pronounced. Body temperature rises to 38°C, intoxication is possible with manifestations of headache, feelings of weakness and myalgia (pain in muscle tissue). Abdominal, articular (inflammatory processes in the joints) and urinary syndromes are expressed.
• III degree - ESR above 40 mm/hour. The patient's condition with this degree of hemorrhagic vasculitis is characterized as severe. Severe intoxication (headache, severe weakness, high fever, myalgia). Abdominal syndrome (pain in the abdomen), articular, nephrotic (inflammatory processes in the kidneys) and skin syndromes are present. Partial disruption of the peripheral or nervous system is possible. There is an increased number of leukocytes and neutrophils in the blood.

Clinical manifestations

The disease is often benign. From the moment of the first rash, in most cases spontaneous recovery occurs within 2-3 weeks. In some cases, a relapse is possible - the disease appears again after a while. Severe complications occur with damage to the kidneys and intestines.

There are several clinical courses of hemorrhagic vasculitis.

The cutaneous form is expressed in all patients. Diffuse rashes of the maculopapular type appear on the skin, which do not disappear with pressure. The size of the rashes is often small and located symmetrically; in some cases, an urticarial (allergic type) rash appears.

The abundance of skin manifestations depends on the severity of the course - in the worst case scenario, necrosis and ulcers may develop at the sites of rash formation. Rashes appear in the area of ​​large joints, thighs, buttocks and lower legs. Rarely - on the arms and torso. With chronic vasculitis, especially if relapses are common, the skin at the site of the rash peels off. Hyperpigmentation is observed.

Articular syndrome appears in 70% of patients. Damage to joint tissue can last for several days or be short-term. Pain in the joints appears, redness and swelling are pronounced. In this form of Henoch-Schönlein disease, symptoms limit the mobility of the joint, making movement very difficult. The largest joints are most often affected, especially the ankles or knees. This syndrome occurs at the beginning or final stage of the disease. Does not lead to chronic joint problems.

The abdominal form precedes or occurs together with the articular form of hemorrhagic vasculitis; symptoms are manifested by moderate or paroxysmal abdominal pain. The patient often cannot indicate the exact location of the pain. Abnormal bowel movements, vomiting and nausea are possible. Signs of pain often resolve spontaneously or within a few days of treatment. In severe cases of the disease, gastrointestinal bleeding may occur with the presence of blood in the vomit and feces.

Renal syndrome is characterized as a course of acute or chronic glomerulonephritis. Occurs in 20-30% of patients. Nephrotic syndrome may develop. May cause kidney failure.

Other organs are rarely affected by the disease. Possible hemorrhagic pneumonia, which occurs with damage to the lungs. Hemorrhagic myocarditis or pericarditis with damage to cardiac tissue. Hemorrhagic meningitis with symptoms of brain damage - headache, light sensitivity, irritability, dizziness appear, and can cause hemorrhagic stroke (bleeding in the brain).

How to diagnose

Diagnosis of Henoch-Schönlein purpura is carried out by a rheumatologist. The patient’s age category, clinical data are taken into account, and laboratory tests are performed. Anamnesis is collected to determine the presence of other diseases. In blood tests in patients with hemorrhagic vasculitis, the inflammatory process and an increased number of platelets and eosinophils are more often expressed.

Urinalysis shows increased protein content, hematuria and cylindruria. An increase in immunoglobulin A and CRP is noted in biochemical blood parameters. A coagulogram is performed to determine blood clotting readings.

If kidney function is impaired, you should consult a nephrologist. They do an ultrasound of the kidneys and an ultrasound scan of its vessels. A urine test is taken for biochemical studies and a Zimnitsky test.

In the case of the abdominal form, other diseases that can cause the same symptomatic signs of an “acute abdomen” are excluded. These include appendicitis, pancreatitis, penetration of gastrointestinal ulcers, cholecystitis, etc. A gastroenterologist surgeon writes out a referral for abdominal ultrasound and gastroscopy. A stool occult blood test is taken to diagnose possible gastrointestinal bleeding.

In severe cases of the disease, a skin biopsy is performed. Histological studies reveal deposits of immunoglobulin A and CIC on the walls of capillaries, venules, arterioles and microthrombosis with blood leaking outside the vessels. Differential diagnosis of hemorrhagic vasculitis is carried out from other diseases that can cause similar symptoms.

How to cure

In the acute phase, when treating hemorrhagic vasculitis, bed rest and a diet with limited allergenic foods are prescribed. You should not take antibiotics or other drugs that increase sensitivity to foreign agents in the blood.

Heparin is the main drug used in the treatment of Henoch-Schönlein disease. Corticosteroids are prescribed, if the measure is ineffective, cytostatics are used. Prednisolone is also used in some cases, but opinions on the effectiveness of the drug for the treatment of Henoch-Schönlein purpura are divided. In severe cases of the disease, immunosorption, hemosorption and plasmapheresis are prescribed.

Treatment of hemorrhagic vasculitis in articular syndrome is carried out by taking anti-inflammatory drugs (Ibuprofen or Indomethacin). Antihistamines are considered ineffective and are used to relieve allergic manifestations. Enterosorbents are prescribed in cases of abdominal disease and food allergies.

Infusion therapy is carried out by diluting the circulating blood and reducing the concentration of possible allergens and immune antibodies. Use with caution in patients with kidney disease. The drugs needed for the procedure to treat the disease are selected individually. Crystalloid solutions are used - concentrated electrolyte with diluted sugars.

Children must be registered with a dispensary for 2 years. For the first 6 months, the patient visits the attending physician every month. After that - once every 3 months, then - once every 6 months. More often, the prognosis for the effectiveness of treatment for hemorrhagic vasculitis is favorable.

Prevention is carried out by sanitizing foci of infection. Stool samples are taken every day to check for the presence of helminth eggs. Children should not undergo physical therapy; physical overexertion and prolonged exposure to the sun are contraindicated. Hemorrhagic vasculitis in adults is milder than in children.