[Clinical characteristics of primary myelofibrosis]

The authors followed-up five patients with primary myelofibrosis in the course of 4 years. The diagnosis, in all cases, was confirmed via histologic investigation of trepenation biopsy material of bone marrow and bone. The clinical and hematologic variety, observed by the authors, provided grounds for the formation of the following variants of the primary myelofibrosis: myelemic-anemic form, form with leucosis transformation, form with sarcomatization, myelofibrosis with immune hemolytic anemia and myelofibrosis in aplastic state. The establishment of transitory forms between the myeloproliferation and dysanaplastic blast processes allows the presumption that the pathogenesis essence of primary myelofibrosis is manifested in clonal lesion of the stem bone-marrow cells.