Dupriez Brigitte, Demory Jean-Loup
Service hématologie clinique, centre hospitalier, 62300 Lens.
Rev Prat. 2005 Oct 15;55(15):1680-5.
Myelofibrosis with myeloid metaplasia is the rarest myeloproliferative syndrom. Diagnosis is often easy in classical form, characterised by splenomegaly, leukoerythroblastic blood reaction and tear-drop erythrocytes on blood count and myelofibrosis on bone marrow biopsy. Evolution is highly variable with a median overal survival of 40 to 60 months and numerous prognostic factors especially anemia. No treatment has been demonstrated to improve survival (apart from allogenic bone marrow transplant). The rarity and the complexity of the disease are the most important difficulties for the definition of standardized diagnostic, prognostic and therapeutic criteria.
骨髓纤维化伴髓外化生是最罕见的骨髓增殖性综合征。典型形式的诊断通常容易，其特征为脾肿大、血常规显示白细胞红细胞系反应和泪滴状红细胞以及骨髓活检显示骨髓纤维化。病情进展差异很大，总中位生存期为40至60个月，有许多预后因素，尤其是贫血。尚无治疗方法被证明可提高生存率（异基因骨髓移植除外）。疾病的罕见性和复杂性是定义标准化诊断、预后和治疗标准的最重要困难。
