home Audience 4 What is essential thrombocythemia, causes and treatment. Essential thrombocythemia, what kind of disease is it and how life-threatening is it?

What is essential thrombocythemia, causes and treatment. Essential thrombocythemia, what kind of disease is it and how life-threatening is it?

Essential thrombocythemia is considered rare, but very insidious disease, which leads to the development dangerous consequences. The article further describes how the pathology proceeds, whether it is oncology or not, whether it can be cured and what the prognosis and complications are.

What it is

The disease in question has other names: essential thrombosis, primary or hemorrhagic thrombocythemia.

This is when the number of platelets in the blood increases, megakaryocytic hyperplasia occurs. This occurs because bone marrow cells called megakaryocytes increase in number and are responsible for producing platelets.

This disease is not oncological, but has some similarities with hemoblastosis - a tumor process in the hematopoietic system.

Symptoms of the pathological condition

In some cases (about a third of patients), the symptoms of the disease are mild.

The disease is usually accompanied by the following pathological conditions:

Hemorrhagic syndrome. It often causes bleeding in the digestive organs, genitourinary system. A clear sign- bleeding from the nose and gums.
Erythromelalgia. It manifests itself as pain that seems to pulsate in the limbs, especially when physical activity. Skin color may change. The patient feels hot.
Cerebrovascular ischemia. In this condition, the patient complains of headache, nausea, and dizziness.

Other symptoms are also observed:

heaviness and pain in the hypochondrium on both sides (as a result of the enlargement of the spleen and liver);
formation of blood clots in veins and arteries;
increased heart rate;
dyspnea;
general weakness;
pale shade skin.

Another sign of the disease is Raynaud's sign, in which necrotic changes and changes are observed in the fingers and toes. pain syndrome due to capillaries becoming clogged with blood clots.

If these symptoms occur, you should consult a specialist.

Causes

The specific causes of primary thrombocythemia have not yet been established. However, a number of experts are inclined to believe that the cause of the pathology may be the body’s hypersensitivity to peptides or a hormone-like protein.

Other scientists believe that possible reason the development of the disease may be reduced sensitivity to components that inhibit cell growth.

According to another hypothesis, essential thrombocythemia develops due to the fact that stem cells at the hematopoietic level begin to mutate.

Secondary thrombocythemia occurs due to the following reasons:

The risk group for the occurrence of the disease is the elderly. However, young people can also develop pathology. Moreover, most often the disease occurs in women.

Diagnostic methods

This pathology is identified by a specialist - a hematologist, who collects anamnesis and examines the patient to determine whether the liver or spleen is enlarged in size, whether there are symptoms of vascular thrombosis and hemorrhage.

To establish a diagnosis, the following diagnostic methods are prescribed:

general blood test (megakaryocytes, increased levels of platelets and leukocytes are detected in the blood);
blood test for coagulation (coagulogram);
cytogenetic and molecular genetic research;
bone marrow tissue tests.

Additional methods for detecting the disease may be:

X-ray of the lungs;
colonoscopy examination;
gastroduodenoscopy;
ultrasound diagnostics.

These methods make it possible to establish thrombocythemia of the secondary form.

Traditional treatment

If the symptoms of the pathology are not pronounced, then no treatment is required, but the patient should be constantly monitored by a doctor. It is recommended to take acetylsalicylic acid, which prevents blood clots.

In case of bright severe symptoms Chemotherapy (cytostatic drugs) is prescribed. This method will reduce the platelet count.

Patients may be prescribed alpha interferon. It is usually prescribed to women when bearing a child, as well as to pediatric patients.

Other drugs used for treatment include hydroxyurea and Anagrelide. However, these drugs are not prescribed during pregnancy.

If the condition threatens the patient's life, then clinical guidelines include plateletpheresis, in which excess platelets are removed by separation.

In case of illness, to improve the patient’s condition and reduce the manifestations of symptoms, one should healthy image life: give up bad habits, eat right, have feasible physical activity.

Alternative medicine

Folk remedies are only auxiliary methods of treatment. That is why they can only be used after the approval of the attending physician.

TO alternative means that are used for the disease include:

Garlic tincture with alcohol. To prepare, chop several heads and pour a glass of vodka. It is recommended to insist for at least 30 days in a place where direct Sun rays. Drink half a teaspoon twice a day.
A decoction of blueberry berries, calamus rhizomes, St. John's wort, Echinops seeds, and celandine. Products taken in equal proportions are boiled in water for ten minutes. It is recommended to drink three times a day.
Infusion of blueberries, agrimony root and burdock, sophora. The ingredients should be taken in equal proportions. They are poured with boiling water and left to cool. Drink a third of a glass three times a day. The duration of treatment is two months.

Horse chestnut is also used to reduce symptoms of the disease. To do this, pour boiling water over the fruits, leaves and flowers and keep them in a water bath for half an hour. Take before meals twice a day. The dosage at one time is a tablespoon.

Nutrition for pathology

Diet has great importance to prevent thrombosis in peripheral blood. Prohibited products in this case are:

Proper nutrition during illness is primarily aimed at providing the body with vitamins, proteins and useful microelements. In addition, it is necessary to consume foods daily that help thin the blood.

dietary meat of chicken, turkey, rabbit;
sea fish;
nuts;
dairy products;
vegetable fats;
eggs;
spices.

The best fruits to eat are citruses, peaches, and apples. Among the vegetables, beets, cabbage (especially when pickled), cucumbers, tomatoes, zucchini, and paprika are considered useful. You are allowed to eat raspberries, wild berries, strawberries and currants.

Disease during pregnancy

When carrying a child, the disease is dangerous due to miscarriage, placental insufficiency and placental abruption.

If the baby is born, his physical and mental development may be delayed.

When pregnancy occurs during the disease, women should use interferon.

Complications and prognosis

A frequent consequence of long-term pathology is fibrosis of bone marrow cells. Sometimes blastic transformation may develop, leading to acute leukemia.

Dangerous complications of the pathological condition include:

thrombosis of blood vessels;
pulmonary embolism;
internal bleeding;
necrotic changes in the tissues of the legs;
enlarged spleen and liver;
growth of lymph nodes.

Experts say that the pathology does not affect the life expectancy of patients, so the prognosis is mostly favorable.

If the patient has pronounced symptoms, he may be given a disability. Depending on the manifestation of the signs, the first, second or third group may be assigned.

Thus, despite the appearance of some symptoms tumor processes, essential thrombocythemia is not cancer. However, it can provoke the development of serious pathologies that can result in death. That is why you should not ignore the symptoms of the disease; it is important to seek help from an experienced hematologist in a timely manner.

Thrombocytosis is a hematological pathology characterized by an increase in the number of platelets in the blood.

The provoking factor is associated with dysfunction of bone marrow stem cells.

Such a disorder causes accelerated “production” of platelets, and becomes an obstacle to decay.

In addition, distribution in the bloodstream “ blood platelets”, as platelets are secondarily called, changes in the direction of increase. The numerical indicator for healthy hematopoiesis ranges from 200-400*10 9 /l. A value that drops below 200 indicates; an increase in values above 400 indicates thrombocytosis.

The list of tasks assigned to these blood cells:

cellular hemostasis
fight against blood clots- dissolving blockages
nutrition, protection of blood vessel walls

Causes of the disease

Determining the type of pathology is an extremely important task, since the disease can appear as an independent “combat-ready unit”, or become a formidable complication of other hematological disorders.

Classify the following types thrombocytosis:

clonal
primary
secondary

The first two items on this list have a similar pathogenesis; the development of pathologies is caused by disorders in hematopoietic stem cells.

In the clonal form, stem cells suffer from tumor damage and differ hypersensitivity to thrombopoietin.

The formation of platelets goes beyond the control of the body; they are produced functionally “defective,” which leads to disruption of normal interaction with other elements of the circulatory system.

Primary thrombocytosis is caused by impaired performance of bone marrow stem cells, in which a single or group proliferation of hematopoietic areas is “fixed”.

Elderly people are at risk.

In children and teenagers, such problems are rare.

Finally, the secondary form of the disease develops against the background of the main chronic pathology disturbing the patient. The etiological pattern is multifaceted:

infection
injuries
inflammation
splenectomy
consequences of surgery
hematological disorders
malignant formations - lymphoma of both types, neuroblastoma, hepatoblastoma
reception medicines: sympathomimetics, corticosteroids, antimitotics

Let's take a closer look at this list.

1. Penetration of infection- this reason occupies a leading position among the factors provoking an increase in platelet levels. Moreover, in the first roles is bacterial, shining example meningococcal disease (pneumonia). Extremely dangerous infectious pathology, widespread, transient. High risk serious complications, even death.

Among other less common reasons, it is worth noting:

2. Stressful state , which occurs after a serious injury or surgery, can become a catalyst for the disease. In addition, the development of pathology is facilitated by tissue damage during enterocolitis.

3. Inflammatory phenomena- a significant factor in the list of reasons that provoke a rapid increase in platelet levels. This increase is due to an increase in the level of interleukin, which promotes the accelerated creation of a hormone called thrombopoietin.

It regulates the life processes of platelets: maturation, division, release into the bloodstream.

List of inflammatory pathologies associated with thickening and increased viscosity blood:

benign lymphogranulomatosis
hemorrhagic vasculitis
acute necrotizing vasculitis
rheumatoid arthritis
collagenosis - similar lesions connective tissue, mostly affecting fibers containing collagen
inflammatory liver problems

4. Hematological factor- insufficient amount of iron. When a symptomatic pattern of thrombocytosis is observed, a ferritin test is certainly prescribed.

5. Splenectomy- a powerful argument for the appearance of reactive thrombocytosis, because being healthy, a third of the platelets produced are localized in this organ. The removal operation entails a reduction in the volume of blood distribution and an artificial increase in the level of blood platelets. Similar condition is inherent in a disease called asplenia - the spleen is absent.

Signs of the disease

People whose age has “crossed” the fifty years mark are at greater risk of becoming acquainted with thrombocytosis, and the gender of the patient does not have a significant impact on the incidence of the disease.

Characteristic manifestations of the disease are the formation of blood clots in blood vessels, increased bleeding.

Veins (uterine, portal, splenic, hepatic) and arteries (pulmonary, carotid, cerebral) can suffer from thrombosis.

As for complaints that are characteristic of elevated platelet levels, the list pathological disorders extensive:

nasal
uterine
gastrointestinal

Besides, symptomatic picture inherent in thrombocytosis can be supplemented by:

itchy skin
cyanosis
tingling of limbs
migraine pain
pressure deviation
dyspnea
the slightest injury provokes the appearance of a bruise
edematous manifestations
the wounds received do not heal well

With systematically recurring bleeding, the development of iron deficiency anemia is likely.

The clinical picture in patients can vary significantly. However, the “popular” symptoms of primary thrombocytosis are: headaches, bleeding.

Diagnosis of thrombocytosis

It's good if correct diagnosis placed before the onset of hemorrhagic disorders and blood clot formation. Started on time adequate treatment, will avoid dangerous complications. They will begin to fight the blood clot initial stage, when the process of platelet aggregation (sticking together of blood cells (cells)) has not gained pathological momentum, exceeding the permissible values. The aggregation rate, indicating good hematopoiesis, “confirming” a complete supply of oxygen to tissues and organs, varies from 30-90%, depending on the inductor.

When held general analysis blood, revealed an increased level of platelets, then the doctor will probably recommend undergoing a specialized examination in a hematology hospital. This scenario is not always physically feasible due to a number of circumstances:

financial opportunities
lack of a specialized medical facility in the locality

Mandatory diagnostic measures, helping to more accurately describe the etiology of the disease:

hematologist conducts an in-depth examination
aspiration biopsy
clinical blood test
trephine biopsy of bone marrow

To exclude an oncological factor, additional studies will be required.

Treatment of thrombocytosis

The vector of direction of the therapeutic process is determined by the type of disease.

In the essential form, the following drugs are included among medical prescriptions:

hydroxyurea
anticoagulants, antiplatelet agents of directed action - means that inhibit, reduce the ability shaped elements(platelets, red blood cells) stick together, stick to each other

These drugs include Heparin, Livarudin, Argotoban.

Systematic (daily) monitoring of platelet levels is required.

Therapy for the clonal form of the disease is based on integrated use antiplatelet drugs. Among these are Aspirin, Clopidogrel, Ticlopidine.

The selection of dosage and duration of administration are determined by the doctor - no independence, initiative is categorically unacceptable.

When making appointments, the patient’s height and weight are taken into account. Regarding acetylsalicylic acid, take into account the probable side effect on the mucous membranes of the gastrointestinal tract, the ulcerogenic effect can provoke relapses of gastritis and ulcers.

Reception is appropriate when there are no intestines, no erosions, ulcerative lesions Gastrointestinal tract. Use only as prescribed by a doctor.

During pregnancy, an increase in platelet levels is more of a physiological event that rarely requires adjustment. Assign special drugs, which, in addition to fighting blood clots, increase immunity and improve uteroplacental blood circulation.

On late stages diseases, when the excess of the norm is noticeable, treatment is expanded due to cytostatic therapy. It is possible to prescribe separation - a procedure to remove excess platelets from the blood. In case of secondary thrombocytosis, the underlying disease that provoked an increase in the number of blood platelets is treated.

Important condition successful treatment- a well-chosen, balanced nutritional diet. They focus on foods enriched with calcium and iron. Pay close attention to B vitamins.

List of authorized products:

Soda and smoked products are not allowed. Reconsider the share of carbohydrate products towards decreasing, because uric acid formed during metabolism is directly related to platelet levels. Porridges, such as buckwheat, bananas, are foods whose consumption needs to be reduced.

Nettle and yarrow are unwanted table guests.

Traditional therapy is considered as a secondary, auxiliary measure. Cocoa, ginger, garlic, cinquefoil, artichoke, and chestnuts are recommended for use. Hirudotherapy is useful, provided the procedure is carried out in an official medical institution.

Excess of formed blood elements, increased viscosity plasma, coagulation - risk factors can lead to the development of thrombophilia. This pathological condition is extremely dangerous, since as a result of blood clotting disorders, blood clots form in blood vessels, mainly venous ones.

Education blood clots due to: physical overload, consequences of surgery, injuries. The sad result is that insufficient blood supply leads to heart attacks of internal organs.

With that said, thrombocytosis is treated with medications that thin the blood and prevent clotting.

Take an interest in health, goodbye.

Essential thrombocythemia (ET) – rare pathology, characterized by a sharp increase in the number of platelets. Formally, it does not apply to oncological diseases blood, however, has tumor lesions hematopoietic system- , some similarities.

What is the disease

Essential thrombocythemia (synonyms: essential thrombocytosis, primary thrombocythemia, hemorrhagic thrombocythemia, chronic megakaryocytic leukemia) – a blood pathology consisting in an increase in the number of platelets. The disease is extremely rare - 2–5 cases per 100,000 population. There is an increase in the number of platelets due to hyperplasia, i.e. an increase in the number of megakaryocytes - especially large cells of the red bone marrow, from which platelets bud, being, in fact, fragments of the cytoplasm of these mother cells, surrounded by their own membrane.

Hematologists note two types of disease - primary and secondary. Primary thrombocythemia is called essential thrombocythemia. The cause of its development is sometimes impossible to establish, while secondary thrombocythemia, as a rule, is a consequence various infections, bleeding, malignant tumor lesions of the body, iron deficiency, rheumatoid arthritis etc. In addition, it can also be the result of removal of the spleen (splenectomy).

At risk for this disease includes women and men over 50 years of age. In men, thrombocythemia is recorded somewhat less frequently than in women.

Reasons for development

As already noted, in some cases it is not possible to determine the cause of essential thrombocythemia, in contrast to the secondary (symptomatic) form of the disease. Experts have established the fact that the process of increasing the number of platelets starts with the beginning pathological changes megakaryocytes. Hematologists hypothesize that megakaryocyte hyperplasia may be caused by increased sensitivity to hormone-like proteins and peptides synthesized by the body (cytokines), or decreased sensitivity in relation to inhibitors - substances that inhibit cell growth.

There is also an opinion that thrombocythemia is a consequence of mutations occurring in hematopoietic stem cells located in bone marrow, from which all blood elements originate. These cells, which have nothing to do with poetry, despite the name, have pluripotency, i.e. the ability to subsequently transform into cells of various tissues and organs. Throughout our lives, hematopoietic stem cells ensure the process of renewing blood cells to replace dead ones.

Symptoms and signs

In approximately 1/3 of patients, clinical manifestations are either vague or absent altogether. In its classic form, the disease manifests itself with a number of main syndromes and signs:

cerebrovascular ischemia - headaches, dizziness, cognitive impairment, nausea, dysfunction of cerebral arteries;
hemorrhagic syndrome - hemorrhages on the skin (petichiae), nosebleeds, bleeding gums, bleeding in the gastrointestinal tract and urinary system;
Raynaud's syndrome - necrotic changes in the extreme phalanges of the upper and upper fingers lower limbs due to capillary thrombosis, accompanied by pain;
erythromelalgia - a feeling of heat and throbbing pain in the arms and legs during physical exertion, a change in the color of the skin;
a feeling of heaviness in both hypochondriums due to enlargement of the liver and spleen;
anemia - weakness, tachycardia, shortness of breath, pallor of the skin and mucous membranes;
thrombosis of blood vessels (usually arteries, less often veins).

For pregnant women, the threat of the disease is the risk spontaneous miscarriage, heart attack and placental abruption, placental insufficiency, leading to developmental delay of the embryo. A child born from such a mother may also exhibit delayed mental, physical and intellectual development.

With a long course, the disease can go into the phase of myelofibrosis (damage to bone marrow cells, 20% of cases) and the phase of blast transformation, causing acute leukemia(2% of cases).

Diagnosis of pathology

To diagnose essential thrombocythemia, you must consult a hematologist. It should be noted that making an accurate differentiated diagnosis in most cases is quite difficult. The following examinations are commonly used:

examination of the patient - determination of enlarged spleen and liver, presence of hemorrhages, signs of vascular thrombosis;
general blood test - allows you to record deviations from the norm of blood components (fragments of megakaryocytes may be present in the blood, increased leukocytosis, a high number of platelets with a change in their shape and size may be noted, and if the hematopoietic process is disrupted, the number of reticulocytes - young erythrocytes - decreases);
- analysis assessing the degree of blood clotting;
laboratory analysis of bone marrow tissue taken through trepanobiopsy and aspiration biopsy(usually from the ilium and sternum) - allows you to detect megakaryocytic hyperplasia;
molecular genetic and cytogenetic studies for the presence gene mutations eg V617F JAK2, JAK2V617F and MPLW515L/K;
additional examinations of the patient (x-ray of the lungs, gastroduodenoscopy, colonoscopy, ultrasound of organs abdominal cavity etc.) - allow you to confirm or refute the presence of symptomatic (secondary) thrombocytosis caused by reasons such as cancerous tumors, bleeding, infections, etc.

To make a differentiated diagnosis, hematologists must take into account a number of formal criteria accepted all over the world:

platelet count > 600,000 per µl in two consecutive blood tests performed 1–2 months apart;
lack of known;
red blood cells are normal;
absence of fibrosis in the bone marrow;
splenomegaly (enlarged spleen);
hypercellularity of the bone marrow with hyperplasia of megakaryocytes;
presence in bone marrow pathological cells in the form of colonies;
normal levels C-reactive protein and interleukin-6;
absence of iron deficiency anemia;
absence of the Philadelphia chromosome;
in women - polymorphism of X chromosome genes.

Treatment methods for essential thrombocythemia

Treatment of pathology involves an integrated approach.

Drug therapy

Most hematologists are of the opinion that in the absence of pronounced clinical picture diseases in special treatment it doesn't need. In this case, it is enough for the patient to be under the supervision of a specialist. Can also be used drug therapy with the use of aspirin, which prevents the formation of blood clots - taking this drug significantly reduces the ability of platelets to adhere to the walls of blood vessels.

In addition to aspirin, a number of other analogues are also used. More information about treatment methods with this and other drugs:

If symptoms are severe, treatment with cytostatic drugs (chemotherapy) can be carried out. In many cases, this therapy can reduce the platelet count to the desired level. Moreover, it should be taken into account that sometimes this type of treatment leads to the transformation of essential thrombocythemia into acute leukemia, and then the only method of treatment remains surgical bone marrow transplantation.

In addition, the use of α-interferon has proven itself well for the treatment of ET. Interferon is widely used to treat pregnant women and children, since it does not have negative impact neither on the embryo nor on the child’s body. But chemotherapy is not usually used in either case due to its high toxicity.

You should know that if you have essential thrombocythemia, you cannot get vaccinations; blood diseases are among the contraindications. The most common example is seasonal influenza vaccination.

Thrombophoresis

When clinical manifestations of the disease pose a danger to the patient’s life, doctors resort to thrombophoresis, a hardware procedure that allows mechanical removal of excess platelets using blood separation. This allows the patient’s condition to significantly improve for some time. In addition, thrombophoresis can be used before emergency surgery to normalize platelet levels.

Diet for increased thrombosis

With ET, the diet should be balanced, capable of providing the body with proteins, vitamins and microelements. Preference should be given to products that can thin the blood. In addition, it is very important to comply drinking regime- the amount of liquid consumed per day should be at least 2–2.5 liters.

lean meats (rabbit, chicken, turkey);
fish, especially sea fish, seafood, seaweed;
eggs;
dairy products;
vegetables - tomatoes, sauerkraut, beets, paprika, cucumbers, zucchini;
fruits - apples, peaches, citrus fruits;
berries - raspberries, currants (red and black), strawberries, wild berries;
walnuts, almonds, cashews;
spices - hot peppers, ginger, garlic, dill, horseradish, cinnamon;
fats - vegetable oils (sunflower, olive, flaxseed), fish oil.

Foods that need to be excluded from the diet, or at least, limit their use:

Prohibited products - photo gallery

Traditional medicine

It should immediately be noted that there is no evidence of the effectiveness of such recipes in the treatment of essential thrombocythemia. Moreover, such treatment can be used only after agreement with the attending physician, and not at the expense of the traditional one.

The following recipes can reduce symptoms and improve the patient’s well-being:

Infusion of blueberries, sophora, burdock roots and agrimony. Mix the ingredients in equal parts, then pour a tablespoon of the raw material into a glass of boiling water, leave, strain and add boiled water to the original volume. Take 1/3 cup three times a day 30 minutes before meals for 1–2 months.
Infusion horse chestnut . To prepare the medicine, take 1 tablespoon of crushed leaves, flowers and fruits of the plant, pour 300 ml of boiling water and keep in a water bath for 10–15 minutes. Then strain the drink and add boiled water to the original volume. Take a tablespoon twice a day half an hour before meals. The course of treatment is 10 days, after which you need to take a 10-day break and repeat again.
A decoction of celandine, St. John's wort, blueberry fruits, Echinops seeds and calamus root. Mix all the ingredients in equal parts, then pour 1 tablespoon of the mixture into an enamel bowl with a glass of boiling water and simmer over low heat for 5–7 minutes, then let the mixture cool, strain it and add boiled water to the original volume. Take the decoction three times a day, one tablespoon half an hour before meals.
Alcohol infusion of garlic. Grind the peeled cloves of 3 medium-sized heads of garlic, pour 250 ml of alcohol or strong vodka into them and leave in a cool, dark place for a month. You need to take the infusion 15 drops twice a day.

Prognosis, possible complications and prevention of the disease

Among the most common complications of essential thrombocythemia:

thrombosis of the coronary, cerebral and peripheral arteries;
pulmonary thromboembolism;
tissue necrosis of the lower extremities due to deep vein thrombosis;
bleeding, including internal;
swollen lymph nodes;
enlarged liver and spleen;
acute leukemia (extremely rare).

Moreover, hematologists believe that the prognosis of the disease is generally favorable, and essential thrombocythemia does not affect the life expectancy of patients. If there are certain pathological signs by decision of the VTEC, the patient may be assigned a disability group - I, II or III (depending on the condition). After the patient’s health improves, the group can be revised or removed.

As for the issue of prevention, unfortunately, such measures are absent today.

The role of platelets in the blood clotting process - video

Essential thrombocythemia is extremely rare, but you should know how this disease can manifest itself. This is necessary in order to promptly consult a specialist, begin therapy and prevent unwanted consequences and complications of this pathology.

Essential thrombocythemia (essential thrombocytosis, primary thrombocythemia) is characterized by an increase in the number of platelets, megakaryocytic hyperplasia and a tendency to bleeding or thrombosis. Patients may complain of weakness, headaches, paresthesia, bleeding; During the examination, splenomegaly may be detected, as well as digital ischemia. Diagnosis is made upon detection increased number platelets (> 500,000/ml), normal quantity red blood cells or normal hematocrit with adequate iron stores, absence of myelofibrosis, Philadelphia chromosome (or ABL-BCR rearrangement), and other diseases that can cause thrombocytosis. There is no single recommended approach to therapy; One of the treatment options is aspirin 81 mg/day orally. In patients over 60 years of age and patients with concomitant diseases To reduce the number of platelets, cytostatic therapy is necessary.

ICD-10 code

D47.3 Essential hemorrhagic thrombocythemia

Epidemiology

Essential thrombocythemia usually occurs between the ages of 50 and 70 years.

Causes of essential thrombocythemia

Chronic inflammatory diseases: RA, inflammatory bowel diseases, tuberculosis, sarcoidosis, Wegener's granulomatosis.
Acute infections.
Bleeding.
Iron deficiency.
Hemolysis.
Tumors: cancer, Hodgkin's lymphoma (Hodgkin's disease), non-Hodgkin's lymphomas.
Surgical interventions(splenectomy).
Myeloproliferative and hematological disorders: polycythemia vera, chronic myeloid leukemia, sideroblastic anemia, myelodysplastic syndrome (5q-cnH-drome), idiopathic myelodysplasia.

Pathogenesis

Essential thrombocythemia (ET) usually develops due to a clonal disorder in a pluripotent hematopoietic stem cell. However, some women who meet the criteria for the diagnosis of essential thrombocythemia have polyclonal hematopoiesis.

With this pathology, increased platelet formation occurs. The lifespan of platelets is within normal limits, although it may be reduced due to sequestration in the spleen. In elderly patients with atherosclerosis, an increase in the number of platelets can lead to severe bleeding or, more often, thrombosis. Bleeding is more common in severe thrombocytosis (platelet level > 1.5 million/μl), which is caused by acquired von Willebrand factor deficiency.

Symptoms of essential thrombocythemia

To the most characteristic manifestations include weakness, bleeding, nonspecific headaches, paresthesia in the hands and feet. Bleeding is usually mild and manifests as nosebleeds, easy education bruises or gastrointestinal bleeding. Ischemia of the fingers is possible; splenomegaly is detected in 60% of patients (the spleen usually does not protrude more than 3 cm from under the edge of the left costal arch). In addition, the development of hepatomegaly is possible. In women, thrombosis can lead to recurrent miscarriage.

Although the disease is usually accompanied by symptoms, its course is generally benign. Severe complications are rare, but can sometimes be life-threatening.

Essential thrombocythemia should be suspected in patients with splenomegaly, as well as in persons with complaints and symptoms characteristic of a myeloproliferative disease, an increase in the number of platelets or disturbances in their morphological structure. If essential thrombocythemia is suspected, a complete blood count, peripheral blood smear, myelogram, and cytogenetic analysis, including determination of the Philadelphia chromosome or BCR-ABL, should be performed. The platelet count may exceed 1,000,000/µl, but may be lower (up to 500,000/µl). Platelet counts often spontaneously decrease during pregnancy. A peripheral blood smear may reveal platelet aggregates, giant platelets, and megakaryocyte fragments. Megakaryocyte hyperplasia and many newly formed platelets are detected in the bone marrow. Iron reserves are stored in the bone marrow. Unlike other myeloproliferative diseases in which thrombocytosis is possible, essential thrombocythemia is characterized by normal hematocrit, MCV and iron levels, as well as the absence of the Philadelphia chromosome and the BCR-ABL translocation (present in chronic myelogenous leukemia), the absence of drop-shaped red blood cells and significant bone marrow fibrosis (as observed in idiopathic myelofibrosis). In addition, this diagnosis requires the exclusion of other pathological conditions, in which secondary thrombocytosis may occur.

Differential diagnosis

Secondary thrombocytosis can develop with chronic inflammatory diseases, acute infection, bleeding, iron deficiency, hemolysis or tumors. Platelet function is usually not impaired. However, in myeloproliferative diseases, platelet aggregation disorders are detected in 50% of patients. Unlike primary thrombocythemia, it does not increase the risk of developing thrombotic or hemorrhagic complications, if patients do not have arterial disease or prolonged immobilization. At secondary thrombocytosis platelet count usually

Treatment of essential thrombocythemia

There is no consensus on when to start therapy. For lungs vasomotor symptoms(For example, headache, mild digital ischemia, erythromelalgia) and in order to reduce the risk of thrombosis in patients from the low-risk group, it is sufficient to prescribe aspirin at a dose of 81 mg x 1 time per day orally. Since the prognosis of the disease is usually good, the use of potentially toxic platelet-lowering therapies should be limited. Patients with severe bleeding require therapy to reduce platelet counts. Patients over 60 years of age with a history of thrombosis or having concomitant pathology who increases the risk of thrombosis should receive drugs that lower platelet levels. The issue of prescribing platelet-lowering drugs in patients under 60 years of age without symptoms of the disease requires further study. Most pregnant women are prescribed aspirin.

This disease is characterized by a significant increase in the content of platelets, the morphology and functions of which are often altered, which is the cause of such clinical manifestations, such as thrombosis and bleeding. Most often, essential thrombocytosis affects middle-aged and elderly people. Manifestations of the disease are very variable; sometimes essential thrombocytosis is discovered by chance in people who do not have clinical symptoms. However, usually the first symptoms of the disease are spontaneous bleeding of varying severity, often occurring in gastrointestinal tract and often recurring over several years. Spontaneous subcutaneous hemorrhages are characteristic.

Thrombosis, usually affecting small vessels, are accompanied by the formation of peripheral ulcers and (or) gangrene, areas of chills and erythromelalgia. A small number of patients experience splenomegaly, sometimes very pronounced and combined with hepatomegaly. Splenic infarctions often occur. The Hess test is usually negative.

Laboratory studies indicate an increase in the number of platelets to 1000-3000·109/l, and the platelets themselves are characterized by morphological and functional disorders, which can explain the paradoxical combination of bleeding and thrombosis. Hemoglobin levels and red blood cell morphology are usually within normal limits unless bleeding has occurred shortly before the study. The white blood cell count is normal. Activity alkaline phosphatase leukocytes are often increased, but the Philadelphia chromosome is absent.

Bleeding time is often prolonged, but clotting time is within normal limits. In the bone marrow, a significant increase in the number and size of megakaryocytes is detected, as well as hyperplasia of the erythroid and myeloid lineages. The disease tends to chronic course with a slow increase in platelet count; Untreated patients may develop myelofibrosis. Death may be due to bleeding, thromboembolism, or another cause unrelated to the underlying disease.

The goal of treatment is to reduce the platelet count to normal values, for which 32P or melphalan is used. Must be entered large dose 32P (370-444 MBq). Asymptomatic patients should also be treated to reduce the risk of bleeding. For thrombotic disorders, acetylsalicylic acid has a good effect.

Attention! The described treatment does not guarantee positive result. For more reliable information, ALWAYS consult a specialist.