摘要
目的:分析9例急性巨核细胞白血病(AMKL)患者的临床资料,并进行文献复习,总结其发病特征、诊断、治疗及预后。方法:根据2008年WHO标准进行诊断,回顾性分析9例AMKL患者的临床及实验室资料,随访预后。结果:9例患者的平均年龄为59(53~68)岁,乳酸脱氢酶均升高,8例患者存在不同程度的贫血,3例患者脾脏肿大,肝脏和淋巴结肿大不多见。骨髓涂片有2例患者原始细胞比例<5%,但流式细胞检查CD41和CD61强阳性,原始细胞>20%。骨髓活检发现7例患者纤维化阳性。染色体核型分析显示6例患者染色体核型异常,5例为复杂核型,2例存在费城染色体。1例患者JAK2V617F突变阳性。9例患者接受阿糖胞苷联合柔红霉素(或去甲氧柔红霉素)诱导治疗,中位生存时间为6(1.1~24)个月。结论:AMKL的诊断需要结合骨髓涂片、骨髓活检、免疫分型或免疫组织化学结果综合判断,AMKL骨髓纤维化比例高、继发性比例高、染色体复杂核型比例高,治疗效果差,是一种预后不良的疾病。
Objective：To analyze the clinical characteristics of 9patients with acute megakaryocytic leukemia（AMKL）,and summarize the characteristics,diagnosis,treatment and prognosis.Method：All patients were diagnosed according to the 2008 WHO diagnostic criteria,the clinical and laboratory data of 9patients with AMKL were analyzed retrospectively,and the prognosis was followed up.Result：The average age of 9patients was 59（53to 68）years,the lactate dehydrogenase of all patients was increased,8cases had various degrees of anemia,3cases had splenomegaly,but hepatomegaly and lymphadenopathy were rare.The percentage of bone marrow blast in 2cases was less than 5%,but CD41 and CD61were strong positive by low cytometry,and the percentage of blast cell was more than 20%.The bone marrow biopsy showed myelofibrosis in 7cases.Chromosome karyotype analysis showed chromosome abnormalities in 6cases,complex karyotype in 5cases and Philadelphia chromosome in 2cases.The JAK2 mutation was positive in 1case.Nine patients were treated with cytarabine combined with daunorubicin（or idarubicin）,the median survival time was 6（1.1to 24）months.Conclusion：The diagnosis of AMKL need to be combined the results of bone marrow smear,bone marrow biopsy with immunophenotype or immunohistochemistry.AMKL is a poor prognosis disease with high percentage of bone marrow fibrosis,secondary disease,complex karyotype and poor therapy effect.
作者
吴宛玲
赵光杰
丁天凌
王小钦
顾静文
WU Wanling ZHAO Guangjie DING Tianling WANG Xiaoqin GU Jingwen(Department of Hematology, Huashan Hospital, Fudan University, Shanghai, 200040, Chin)
出处
《临床血液学杂志》
CAS
2017年第5期701-704,共4页
Journal of Clinical Hematology
基金
上海市第四轮公共卫生三年行动计划重点学科建设项目(No:15GWZK0801)
上海市科研计划资助项目(No:16ZR1404400)
关键词
急性巨核细胞白血病
诊断
预后
acute megakaryocytic leukemia
diagnosis
prognosis
