Trombotsitemiya

The essential trombotsitemiya is the chronic megakariotsitarny leukosis relating to myeloproliferative diseases. Stem cells in marrow are involved in process. The disease is by the nature a hemoblastosis, that is tumoral. Uncontrolledly the quantity of megacaryocytes, and then thrombocytes increases. The essential trombotsitemiya meets rather seldom. Prevalence makes 3-4 cases on 100 thousand of adult population. Aged people of 50-60 years are more subject to this disease. Women are ill slightly more often than men. As well as for any oncological disease, exact origins of an essential trombotsitemiya are unknown. Communication with radiation defeat of the environment is noted. The role and other factors is not excluded.

Trombotsitemiya symptoms

The disease is characterized by a long current without visible manifestations. Progressing of a disease slow. Often from the first recorded changes in blood tests before emergence of the first complaints there pass months, and even years. Symptoms of a trombotsitemiya consist of simultaneous tendency to formation of blood clots and bleedings. The origins of these phenomena include disturbances from aggregation of thrombocytes (both towards increase, and towards decrease). Cerebral, coronary and peripheral fibrinferments of arteries are characteristic. At an essential trombotsitemiya the thromboembolism of a pulmonary artery and a deep vein thrombosis of legs are possible. From bleedings gastrointestinal, pulmonary, renal, and also skin hemorrhages meet more often. Besides, at a trombotsitemiya increase in a spleen and liver can develop. These symptoms happen at 50 and 20 percent of patients respectively. The numbness and decrease in sensitivity in fingers of hands and legs, lobes of ears, a nose tip connected with disturbance of blood circulation in small vessels are observed. At a part of patients pains in podreberye and on the intestines course are possible. Many patients lose body weight. Sometimes there are increased groups of lymph nodes. It is a lot of nonspecific symptoms of a trombotsitemiya: general weakness, headache, decrease in working capacity, fatigue, skin itch, frequent change of mood, fervescence.

Diagnosis of a trombotsitemiya

Diagnosis of a disease begins with registration of a large number of thrombocytes in the general blood test. The diagnosis is made at a thrombocytosis over 600 thousand in мкл after an exception of reactive thrombosis. Thrombocytes have different degree of functional inferiority. A prothrombin time, the activated partial tromboplastinovy time, a bleeding time, time of life of platelets within norm. In marrow on results of a puncture the increased cellularity and мегакариоцитоз is found. Cells predecessors of thrombocytes have the huge size, displastichna. Not strictly specific genetic anomalies at an essential trombotsitemii-it mutation JAK2V617F and MPLW515L/K.

Differential diagnosis of a trombotsitemiya and secondary thrombosis

It is difficult to distinguish an essential trombotsitemiya from the secondary thrombosis caused by an amyloidosis, an infection, oncology or other factors. The American board of hematologists developed the following criteria of differential diagnosis:

- quantity of thrombocytes more than 600 000 in мкл in two sequential analyses of blood made with an interval of 1 month;

- lack of the known reason of a reactive thrombocytosis;

- normal quantity of erythrocytes;

- absence of considerable fibrosis in marrow;

- lack of the Philadelphian chromosome;

- increase in a spleen;

- hyper cellularity of marrow with a hyperplasia of megacaryocytes;

- existence in marrow of pathological cells in the form of colonies;

- normal levels of S-reactive protein and interleykina-6;

- lack of an iron deficiency anemia;

- women have a polymorphism of genes of X-chromosome.

The more it is found compliances, the it is more than data in favor of an essential trombotsitemiya.

Treatment of a trombotsitemiya

If at the patient the essential trombotsitemiya comes to light, then to him individual treatment according to the existing scheme has to be picked up. Therapy by that who has risk of thromboses is especially intensively appointed. It is possible to carry the patients of advanced age, patients with a diabetes mellitus, a hypertension having a dislipidemiya to such patients. Treatment of a trombotsitemiya is most often shown to such patients by cytostatics. These are the chemotherapeutic drugs reducing activity of cell fission. Long ago and with success for treatment of a trombotsitemiya hydroxurea is used (0,5-4 g in daily). Because of ability to cause leukoses (for example, an acute miyeloblastny leukosis) this drug is not appointed to children.

Therapy of a trombotsitemiya can be carried out by means of interferon alpha. It is used especially widely at pregnant women as has no the damaging effect on a fruit. In general, restriction for purpose of interferon alpha are its high cost and bad portability. The initial dose of medicine of 1 million ME three times a week, further a dose raises to 3-6 million ME three times a week. About 20% of patients are forced to stop treatment as they are strongly disturbed fervescence, a joint pain and muscles, nausea, appetite loss, grippopodobny symptomatology.

Also for treatment of a trombotsitemiya use анагрелид. This drug selectively slows down maturing of megacaryocytes, influencing other sprouts of a hemopoiesis a little. An initial dose of drug of 2 mg a day, maximum – 10 mg. Medicine has the side effects connected with cardiovascular system. Vasodilatation and increase of heartbeat, hypostases are characteristic. If the patient already has any pathology of heart, then it is not desirable for it to appoint анагрелид. Under the influence of drug there can be a transformation of a trombotsitemiya in a myelofibrosis. Because of all these phenomena анагрелид it is applied generally at intolerance of hydroxurea and interferon alpha.

Perhaps successful use of a plateletpheresis in combination with acetylsalicylic acid (325 mg inside in days) for prevention of thromboses at a trombotsitemiya.

Treatment of an essential trombotsitemiya folk remedies

Folk remedies are sometimes applied by patients at treatment of this disease. No researches on the national methods which proved their efficiency exist. Any such methods are applied by the patient at own risk. Most likely, positive shifts are connected with effect of placebo, that is auto-suggestion. It is essentially important to patient to report to the attending physician about what is still applied independently to fight against an illness. Treatment of an essential trombotsitemiya folk remedies includes use of starvation and various phytodrugs. Most often recommend infusions of fruits of bilberry, seeds of a globe-thistle and train broth.