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Myelofibrosis

Myelofibrosis — a myeloproliferative disease of which are characteristic anemia of various expressiveness — from the moderated to heavy, very various changes in peripheral blood, fibrosis of marrow and a myeloid metaplasia of a spleen, liver and other bodies. The illness results from proliferation of the mutant clone coming from the stem hemopoietic cell capable to be differentiated in the direction of erythrocytes, granulocytes and thrombocytes. Also proliferation of osteoblasts and formation of a new bone tissue is quite often observed. It is unknown whether marrow fibrosis is reaction to disturbances of proliferative activity of cells of the hemopoietic system or a component of such pathological proliferative reactions. The myeloid metaplasia of a spleen, liver and other bodies usually is considered as compensatory process; it is possible, however, that it is result of proliferation of a stem cell.

Clinical manifestations of a myelofibrosis

The myelofibrosis can arise against an Osler's disease or a trombotsitemiya, however usually it develops as primary process. It is necessary to emphasize once again that it is an illness of people of middle and advanced age: the maximum incidence is noted in an age group of 50 — 70 years. Persons of both sexes are ill equally often.

The illness progresses slowly, and because of the hidden beginning the diagnosis is often made at inspection of the patient in absolutely other occasion. The symptoms caused by anemia are often observed: drowsiness, weakness and an asthma at an exercise stress. Sometimes the symptoms caused by increase in a spleen namely abdominal distention, a sensation of discomfort after meal, heartburn and even hypostases in anklebones happen the first displays of a disease. The splenomegaly — almost constant sign, a spleen in one cases is slightly increased, in others reaches the huge sizes, actually occupying all abdominal cavity. The heart attack of a spleen is followed by an acute pain in left hypochondrium and a peritoneum friction murmur. Often the hepatomegalia comes to light. Portal hypertensia results from thrombosis of a splenic vein, formation of extramedullary infiltrates of the proliferating cells on the course of a portal path or its increased krovenapolneniye. Patients with portal hypertensia can have bleedings, a varicosity of a gullet and ascites.
Quite often symptoms of gout are found. Patients also suffer from an itch which becomes especially painful in heat. There are spasms in gastrocnemius muscles, ostealgias, and all these symptoms it is very easily possible to charge to age of the patient.

The functional disturbances of thrombocytes which are found sometimes happen the reason of intradermal hemorrhages and gastrointestinal bleedings. Approximately the focal osteosclerosis which is usually taking bones of an axial skeleton and a proximal epiphysis humeral and femurs is found in 1/3 patients with a myelofibrosis. Occasionally also other sites of a skeleton, for example a skull are surprised.

Datas of laboratory

By the time of the request for medical care anemia is found in 2/3 all patients. Being poorly or moderately expressed at early stages of a disease, in process of its progressing it becomes heavier. The polychromatophilia, an anisocytosis, a poikilocytosis are noted, cells of teardrop shape appear. In peripheral blood yadrosoderzhashchy erythrocytes and unripe granulocytes quite often meet. Often there is a deficit of folic acid caused by insufficient receipt it with food and the raised turn of blood cells. In case of blood losses there is a deficit of iron. The number of leukocytes is often increased, they are provided mainly by mature leukocytes, also unripe forms usually meet.

The number of thrombocytes can be reduced or increased depending on a disease stage. On early phases their number can reach 1000·109/l. The morphology of thrombocytes is changed, in a smear of peripheral blood huge forms and fragments of the circulating megacaryocytes meet. In process of progressing of an illness and increase in a spleen the number of thrombocytes decreases.

Punctures of marrow are usually unsuccessful ("a dry puncture"), however because of focal nature of defeats at a myelofibrosis sometimes it is possible to receive punctates of giperplazirovanny sites. For exact diagnosis it is necessary to carry out a trepanobiopsiya of an ileal bone. Activity of the hemopoietic cells and degree of marrowy fibrosis are variable. With the help" a silvering method increase in amount of reticulin fibers manages to be found even in giperplazirovanny fragments.
Level of uric acid in blood at a myelofibrosis is usually high.

Current and forecast of a myelofibrosis

The condition of many patients for many years remains stable at the normal level of hemoglobin and the minimum splenomegaly. At some patients the disease proceeds is less benign, is characterized by a gradual aggravation of symptoms of health, progressing of anemia and splenomegaly. The average term of life from the moment of establishment of the diagnosis makes 3 years, but many patients live longer. Adverse predictive signs are heavy anemia which does not manage to be eliminated by means of hemotransfusions, the expressed leukopenia, spontaneous bleedings and fast loss of body weight. All these symptoms can be caused by deficit of folic acid. Most often the progressing anemia is a cause of death, about 20% of patients die of an acute miyeloblastny leukosis.

Treatment of a myelofibrosis

There is no specific treatment. Patients with moderately expressed symptoms need only periodic inspection. Anemia is the main occasion to start therapy. Quite often arising deficit of folates well will respond to treatment folic acid. A myelofibrosis — one of the few diseases at which long preventive reception of folic acid in a dose of 5 mg/days is shown. It is necessary to be convinced that at the patient B12 vitamin absorption is not broken. Many patient appoint androgens, however the last are not always effective, improvement of a state if comes, then only several weeks later.
If anemia is so heavy that it causes cardiovascular disturbances, then it is necessary to transfuse blood, trying to support hemoglobin at the level of 90 — 100 g/l. However before starting the program of repeated hemotransfusions, it is necessary to estimate the general condition of the patient attentively. After hemotransfusion rise in level of hemoglobin often happens less expected, and finally its gain becomes less in size well on time, and improvement of a state lasts only 1 — 2 week. It can be caused by destruction of the poured erythrocytes in a spleen.

Deposition of blood in a spleen is the important reason of development of anemia, extent of deposition needs to be defined with the help 51Cr. Very effective remedy of reduction of the sizes of a spleen is Myelosanum — the short course of treatment this drug leads to long improvement of a condition of the patient.
For reduction of the sizes of a spleen use also its radiation which results are ambiguous. It is not recommended to resort to a splenectomy at patients of advanced age because of a high lethality and numerous complications, and also because of possible reactive increase in maintenance of thrombocytes. The embolization method through an intra arterial catheter is safer.
Treatment of patients with a hyperuricemia is described above.

 
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