Essential thrombocythemia - it
chronic megakaryocytic leukemia, myeloproliferative diseases related to. The process involves the stem cells in the bone marrow. The disease is inherently hemoblastosis, i.e. tumor. Unchecked increases the number of megakaryocytes, and then platelets. Essential thrombocythemia is quite rare. The prevalence of 3-4 cases per 100,000 adult population. More prone to this disease people aged 50-60 years. Women suffer more often than men. As with any cancer, the exact causes of essential thrombocythemia is unknown. There connection with radiation damage to the surrounding environment. Not exclude the role of other factors.
The symptoms of thrombocythemia
The disease is characterized by a prolonged course with no visible manifestations
. The slow progression of the disease
. Most of the first documented changes in blood tests before the first complaints are months or even years
. The symptoms of thrombocythemia consist of simultaneous tendency to form blood clots and bleeding
. The mechanism of occurrence of these phenomena include violations by platelet aggregation (in the upward and downward)
. Characteristic cerebral, coronary and peripheral arterial thrombosis
. In essential thrombocythemia possible pulmonary embolism and deep vein thrombosis leg
. From bleedings occur increasingly gastrointestinal, lung, kidney, and skin hemorrhages
. Furthermore, when thrombocythemia may develop liver and spleen enlargement
. These symptoms are 50 and 20 percent of patients, respectively,
. Observed decrease in sensitivity and numbness in the fingers and toes, earlobes, tip of the nose associated with the violation of blood circulation in the small vessels
. Some patients may be pain in hypochondria and in the course of the intestine
. Many patients lose weight
. Sometimes there are enlarged lymph node groups
. Many non-specific symptoms of thrombocythemia: general weakness, headache, reduced disability, fatigue, itchy skin, frequent changes of mood, increased body temperature
Diagnosis of the disease begins with the registration of a large number of platelets in the blood count. Diagnosis is made when thrombocytosis over 600 thousand in l after excluding jet thrombosis. Platelets have a different degree of functional disability. Prothrombin time, activated partial thromboplastin time, bleeding time, the lifetime of the platelets in the normal range. The results of the bone marrow puncture showed increased cellularity and megakariotsitoz. Platelet precursor cells have the size of a giant, dysplastic. Not strictly specific genetic abnormalities in essential trombotsitemii- is JAK2V617F and MPLW515L / K mutation.
Differential diagnosis of thrombocythemia and secondary thrombosis
It is difficult to distinguish from essential thrombocythemia secondary thrombosis, caused by amyloidosis, infection, cancer, or other factors. The American College of Hematology has developed the following criteria for the differential diagnosis:
- Platelet count of more than 600 000 L in two consecutive blood analyzes performed at intervals of 1 month;
- The absence of a known cause of reactive thrombocytosis;
- Normal number of red blood cells;
- No significant fibrosis in the bone marrow;
- The absence of the Philadelphia chromosome;
- Enlargement of the spleen;
- Hypercellularity of bone marrow hyperplasia of megakaryocytes;
- The presence of pathological bone marrow cells as colonies;
- Normal levels of C-reactive protein and interleukin-6;
- The absence of iron deficiency anemia;
- Women - polymorphisms of genes of the X chromosome.
The more matches found, the more data in favor of essential thrombocythemia.
Treatment for thrombocythemia
patient revealed essential thrombocythemia, he should be selected individual treatment in accordance with the existing scheme. Particularly intensive therapy is assigned to those who have the risk of thrombosis. Such patients include elderly patients, patients with diabetes, hypertension, dyslipidemia with. Such patients often show cytostatic treatment of thrombocythemia. This chemotherapeutic drugs that reduce activity of cell division. It has long and successfully used for the treatment of thrombocythemia hydroxyurea (0, 5-4 g orally daily). Due to the ability to cause leukemia (eg, acute myeloid leukemia), this drug is not prescribed to children.
Therapy thrombocythemia may be conducted using interferon alpha. It is used widely especially in pregnant women, as it does not have a damaging effect on the fetus. In general, the restriction for assignment of interferon-alpha are its high cost and poor portability. The initial dose of drug 1 million. IU three times a week, then the dose is increased to 3-6 million. IU three times per week. About 20% of patients are forced to discontinue treatment, since they strongly disturb fever, joint pain and muscle pain, nausea, loss of appetite, flu-like symptoms.
It is also used for the treatment of thrombocythemia anagrelide. This drug selectively inhibits the maturation of megakaryocytes, little effect on the other shoots hematopoiesis. The initial dose is 2 mg per day, maximum - 10 mg. The drug has side-effects associated with the cardiovascular system. Characterized by vasodilatation and heart palpitations, edema. If the patient already has any abnormality of the heart, then it is not advisable to appoint anagrelide. Under the influence of the drug may occur in the transformation of thrombocythemia myelofibrosis. Because of all these phenomena anagrelide is used mainly in case of intolerance hydroxyurea and interferon-alpha.
Possibly successful application trombafereza in combination with acetylsalicylic acid (325 mg orally daily) to prevent thrombosis in thrombocythemia.
Treatment of essential thrombocythemia folk remedies
Folk remedies are sometimes used by patients in the treatment of this disease. No studies on national practices, proven their effectiveness, does not exist. Any such methods are applied by the patient at your own risk. Most likely, the positive changes related to the placebo effect, that is, auto-suggestion. The patient is fundamentally important to inform your doctor about what else is used alone to combat the disease. Treatment of essential thrombocythemia folk remedies include the use of starvation and various herbal remedies. Most often recommend bilberry extracts, seeds Echinops and broth succession.