伴成熟浆细胞样树突细胞增生的骨髓增生异常综合征一例并文献复习  

Myelodysplastic syndromes combined with mature plasmacytoid dendritic cells proliferation:report of one case and review of literature

作　　者：刘静[1] 阎洪敏[1] 朱玲[1] 郑晓丽[1] 丁丽[1] 韩冬梅[1] 谭希同 王恒湘[1] 薛梅[1] Liu Jing;Yan Hongmin;Zhu Ling;Zheng Xiaoli;Ding Li;Han Dongmei;Tan Xitong;Wang Hengxiang;Xue Mei(Department of Hematology, Air Force Medical Center (Former General Hospital of Air Force), Beijing 100142, China)

机构地区：[1]空军特色医学中心(原空军总医院)血液科,北京100142

出　　处：《白血病．淋巴瘤》2019年第7期401-403,共3页Journal of Leukemia & Lymphoma

摘　　要：目的探讨伴成熟浆细胞样树突细胞(PDC)增生的骨髓增生异常综合征(MDS)患者的临床特点、诊断、治疗及预后.方法回顾性分析空军特色医学中心诊治的1例伴成熟PDC增生的MDS伴原始细胞增多(EB)﹣1患者的临床资料,并进行相关文献复习.结果该例患者体检发现贫血及血小板减少,骨髓涂片可见原始粒细胞占0.064,树突细胞占0.152,免疫分型可见原始粒细胞及成熟PDC两组异常增殖的细胞,给予地西他滨治疗,间断输注红细胞及血小板,病情基本稳定.结论伴成熟PDC增生的MDS极为罕见,临床无特殊表现,诊断依据骨髓细胞学检查及免疫分型,治疗尚无标准方案,预后依据MDS疾病进展而定.Objective To investigate the clinical features,diagnosis,treatment and prognosis of myelodysplastic syndromes(MDS)with mature plasmacytoid dendritic cells(PDC)proliferation.Methods The clinical data of one case of MDS with excess blasts(EB)-1 with mature PDC proliferation in Air Force Medical Center was retrospectively analyzed,and the literature was reviewed.Results The patient's physical examination revealed anemia and thrombocytopenia.Bone marrow smears showed 0.064 of myeloblasts and 0.152 of dendritic cells.Immunophenotyping showed two groups of abnormal proliferation cells,namely,myeloblasts and mature PDC.Decitabine treatment was given,and the red blood cells and platelets were infused intermittently.The condition of patient was basically stable.Conclusions MDS with mature PDC proliferation is extremely rare.No special clinical manifestations are found,and the diagnosis is based on bone marrow cytology and immunophenotyping.There is no standard regimen for treatment of MDS with mature PDC proliferation,and the prognosis depends on the progression of MDS.

关 键 词：骨髓增生异常综合征 浆细胞样树突细胞 诊断 治疗 

分 类 号：R551.3[医药卫生—血液循环系统疾病]