中文摘要：

目的分析原发性骨髓增生异常综合征伴骨髓纤维化（MDS-MF）患者的临床特征和预后，提高对MDS-MF的认识。方法回顾性分析有骨髓活检结果的466例原发性MDS患者资料，根据是否伴有MF分为两组比较临床特征，并进行预后分析。结果466例原发性MDS患者中167例（35．8％）MDS患者伴有MF，其中MF-1级123例（26．4％），MF-2级40例（8．6％），MF-3级4例（0．9％）。MDS-MF组患者肝脏和（或）脾脏肿大比例高于MDS不伴MF组，差异有统计学意义（P=0．031）。MDS-MF组患者骨髓活检增生程度较MDS不伴MF组活跃，MDS-MF组患者骨髓原始细胞数、巨核细胞数和异常巨核细胞数高于MDS不伴MF组，差异均有统计学意义（P值均〈0．05）。有可供分析的细胞遗传学检查结果的345例患者中MDS-MF患者121例，MDS-MF组患者染色体核型预后分组中危及高危组比例高于MDS不伴MF组，差异有统计学意义（P=0．047）。MDS-MF组患者中位生存期17（1-60）个月，MDS不伴MF组患者中位生存期32（1-62）个月，两组差异有统计学意义（P=0．001）。将单因素分析有预后意义的参数纳入COX模型进行多因素分析，结果显示MF具有独立预后意义（P=0．019）。结论MDS伴MF患者骨髓以网状纤维增生为主，其网状纤维增生与原始细胞数、巨核细胞的增生和发育异常、染色体核型密切相关。伴有MF的MDS患者预后较差。

英文摘要：

Objective To analyze the clinical features and prognosis of the primary myelodysplastic syndrome with myelofibrosis （MDS-MF） patients and to improve the cognition of MDS-MF. Methods Four hundred and sixty-six primary MDS patients with bone marrow（BM） biopsy were divided into two groups ac- cording to whether BM associated with fibrosis, the clinical features and prognosis of the two groups were ana- lyzed retrospectively. Results 167 （35.8%） MDS cases revealed myelofibrosis, of which MF-1 123 cases （26.4%） , MF-2 40 cases （8.6%） , MF-3 4 cases （ 0.9% ）. The proportion of hepatosplenomegaly in MDS-MF group was significantly higher than in MDS without MF group, the difference had statistical signifi- cance （P =0.031 ）. The proliferation of BM biopsy in MDS-MF group was significantly more active than in MDS without MF group. The number of blasts, megakaryocytes and abnormal megakaryocytes in MDS-MF group were significantly higher than in MDS without MF group, the differences had statistical significance （P 〈 0. 05 ）. Among the 345 patients who had available results of cytogenetic analysis, 121 cases were MDS- MF patients, the proportion of middle and high-risk prognostic group according to IPSS karyotype prognosis groups in MDS-MF group were significantly higher than in MDS without MF group, the differences had statisti- cal significance （ P = 0. 047 ）. The median survival was 17 （ 1 - 60 ） months in MDS-MF group, and was 32 （1 -62 ） months in MDS without MF group. The difference had statistical significance （P = 0. 001 ）. Myelo- fibrosis had independent prognostic significance by multi-variable analysis （ P = 0. 019 ）. Conclusion The myelofibrosis in MDS is main the proliferation of reticular fiber. The proliferation of reticular fiber is closely related with the number of blast cells, the proliferation and developmental abnormalities of megakaryocytesand the karyotype. The prognosis of MDS-MF patients is poor.