Hasselbalch H C
Department of Medicine, Naestved Hospital, Denmark.
Int J Clin Lab Res. 1993;23(3):124-38. doi: 10.1007/BF02592297.
Idiopathic myelofibrosis (IMF) is characterized by excessive accumulation of connective tissue in the bone marrow as part of a clinical syndrome which in its classical form is featured by leukoerythroblastic anemia and huge splenomegaly at the time of diagnosis. An acute variant of the disease exists being featured by pancytopenia, nor or minimal splenomegaly and a rapidly fatal clinical course. This review describes the relationship of IMF to other chronic myeloproliferative disorders and highlights current concepts of the pathogenesis of bone marrow fibrosis, implicating the intramedullary release of various growth factors, including platelet-derived growth factor beta. In a subgroup of patients bone marrow fibrosis may develop consequent to autoimmune bone marrow damage. The clinical and laboratory findings in some of the larger series of patients are presented and the reasons for the highly variable clinical presentation and prognosis are critically discussed. It is proposed that studies on prognosis in IMF are based upon simple prognostic staging systems, which should include the Hb-concentration, platelet count, spleen size and the presence/absence of osteomyelosclerosis on X-ray. Using these parameters the patients are easily categorized into three prognostic groups with highly different survival times.
原发性骨髓纤维化（IMF）的特征是骨髓中结缔组织过度积聚，这是一种临床综合征的一部分，其典型形式在诊断时以白细胞红细胞贫血和巨大脾肿大为特征。该疾病存在急性变异型，其特征为全血细胞减少、无脾肿大或脾肿大极小以及临床病程迅速致命。本综述描述了IMF与其他慢性骨髓增殖性疾病的关系，并强调了目前关于骨髓纤维化发病机制的概念，涉及包括血小板衍生生长因子β在内的各种生长因子的髓内释放。在一部分患者中，骨髓纤维化可能继发于自身免疫性骨髓损伤。文中展示了一些较大系列患者的临床和实验室检查结果，并对临床表现和预后高度可变的原因进行了批判性讨论。有人提出，关于IMF预后的研究应基于简单的预后分期系统，该系统应包括血红蛋白浓度、血小板计数、脾脏大小以及X线检查中是否存在骨髓硬化。使用这些参数，患者很容易被分为三个预后组，其生存时间差异很大。
