Simeonov Ts, Petrov I
Vutr Boles. 1979;18(6):108-12.
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.
作者在4年的时间里对5例原发性骨髓纤维化患者进行了随访。所有病例均通过骨髓和骨骼穿刺活检材料的组织学检查得以确诊。作者观察到的临床和血液学变化为原发性骨髓纤维化的以下几种变体的形成提供了依据：髓性贫血型、伴有白血病转化型、伴有肉瘤样变型、伴有免疫性溶血性贫血的骨髓纤维化以及再生障碍状态下的骨髓纤维化。骨髓增殖与发育异常的原始细胞过程之间过渡形式的确定，使得人们推测原发性骨髓纤维化的发病机制本质表现为骨髓干细胞的克隆性病变。
