费城染色体阴性骨髓增殖性肿瘤转化为急性白血病八例并文献复习  

Acute leukemia transformed from myeloproliferatlve neoplasms with Philadelphia chromosome-negative： report of eight cases and review of literature

作　　者：毛原飞[1] 陈秋生[1] 陈瑜[1] 李军民[1] 吴文[1] Mao Yuanfei;Chen Qiusheng;Chert Yu;Li Junmin;Wu Wen(Department of Hematology,Ru~＇in Hospital Affiliated of Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China)

机构地区：[1]上海交通大学医学院附属瑞金医院血液科,上海200025

出　　处：《白血病．淋巴瘤》2018年第8期474-478,共5页Journal of Leukemia & Lymphoma

摘　　要：目的分析费城染色体阴性（Ph一）的骨髓增殖性肿瘤（MPN）转化为急性白血病（AL）的疾病进展过程，并探讨其发病机制及治疗情况。方法回顾性分析上海交通大学医学院附属瑞金医院2013年7月至2017年12月收治8例Ph—MPN转化为AL患者转化前后的临床资料，同时对相关文献进行复习。结果8例MPN患者均转化为急性髓系白血病（AML），中位转化时间为47．5个月（2．180个月），转化后中位生存时间为2个月（1～17个月）。4例转化后行AML相关分子生物学检查的患者中3例出现新的突变基因。1例难治患者口服芦可替尼治疗后疾病稳定。结论MPN转化为AML的患者临床预后差，生存期短。异基因造血干细胞移植是唯一已知的潜在治愈策略，JAK2抑制剂可能有效。Objective To analyze the disease progression of acute leukemia （AL） transformed from myeloproliferative neoplasms （MPN） with Philadelphia chromosome-negative （Ph-）, and to investigate its mechanism and clinical treatments. Methods The pre-transformation and post-transformation data of 8 AL patients with Ph- MPN from July 2013 to December 2017 in Ruijin Hospital Affiliated of Shanghai Jiao Tong University School of Medicine were retrospectively analyzed. The literature was also reviewed. Results All 8 cases transformed into acute myelogenous leukemia （AML）. The median conversion time was 47.5 months （2-180 months）, and the median survival time after transformation was 2 months （1-I7 months）. Three of 4 patients who undergone AML-related molecular biology after transformation had new mutant genes. One refractory patient achieved stable disease after oral treatment with ruxolitinib. Conclusions AML patients transformed from MPN have poor clinical outcomes and short survival time. Allogeneic hematopoietic stem cell transplantation is the only known potential curative treatment strategy and JAK2 inhibitor may be effective.

关 键 词：骨髓增殖性疾病 白血病 髓样 急性 治疗 预后 

分 类 号：R733.71[医药卫生—肿瘤]