以骨髓增殖性肿瘤为首发表现的T淋巴母细胞淋巴瘤合并急性髓系白血病1例并文献复习  

Myeloproliferative neoplasm as the first manifestation in T-lymphoblastic lymphoma with progression to acute myelocytic leukemia:a case report and literature review

作　　者：陈枫煜[1] 裴强[1] 王启 姚翔媚 CHEN Fengyu;PEI Qiang;WANG Qi;YAO Xiangmei(Department of Hematology,the First People′s Hospital of Yunnan Province,Kunming,Yunnan 650032,China)

机构地区：[1]云南省第一人民医院血液科,昆明650032

出　　处：《重庆医学》2020年第14期2373-2376,2379,共5页Chongqing medicine

摘　　要：目的探讨以骨髓增殖性肿瘤(MPN)为首发表现的T淋巴母细胞淋巴瘤(T-LBL)合并急性髓系白血病(AML)的诊断和治疗。方法分析1例以MPN为首发表现的T-LBL患者合并AML的诊治过程,包括病史及入院检查、诊断思路、病理及细胞遗传学检测、治疗及疗效等资料,并进行相关文献复习。结果该患者初诊考虑为MPN,经淋巴结活检确诊为T-LBL,经联合化疗获得部分疗效,后合并AML。结论T-LBL的临床表现可多种多样,病理诊断仍是淋巴瘤诊断的金标准,需全面分析临床表现,予以正确的诊断与治疗,合并AML者罕见,预后差。Objective To explore the diagnosis and treatment of T-lymphoblastic lymphoma(T-LBL)with progression to acute myelocytic leukemia(AML)firstly manifestated by myeloproliferative neoplasm(MPN).Methods The processes of diagnosis and treatment of a T-LBL patient with progression to AML firstly manifestated by MPN,including medical history and admission examination,diagnosis ideas,pathological and cytogenetic detections,and treatment and its outcomes,were analyzed.The related literatures were reviewed,as well.Results This patient was initially considered as MPN,and was finally diagnosed as T-LBL by lymph node biopsy.After combined chemotherapy,this patient required partially remission.As a result,the patient underwent subsequent AML.Conclusion There are varied clinical manifestations in T-LBL.The pathological diagnosis is still the gold standard for the diagnosis of lymphoma.It is necessary to comprehensively analyze the clinical manifestations and give correct diagnosis and treatment.Those patients who progress to AML are rare,and have a poor prognosis.

关 键 词：骨髓增殖性肿瘤 淋巴母细胞淋巴瘤 急性髓系白血病 嗜酸性粒细胞增多 8p11骨髓增生综合征 

分 类 号：R551[医药卫生—血液循环系统疾病]