早期前T淋巴母细胞白血病/淋巴瘤2例并文献复习  

Early T-cell Precursor Acute Lymphoblastic Leukemia/Lymphoma:Two Case Reports and Literature Review

作　　者：盛俊杰 刘聪艳[1] 万岁桂[1] SHENG Junjie;LIU Congyan;WAN Suigui(Department of Hematology,Xuanwu Hospital of Capital Medical University,Beijing,100053,China;Department of Hematology,the Fourth Hospital of Wuhan,Wuhan 430033,China)

机构地区：[1]首都医科大学宣武医院血液科,北京100053 [2]武汉市第四医院血液科,湖北武汉430033

出　　处：《标记免疫分析与临床》2020年第10期1663-1667,1672,共6页Labeled Immunoassays and Clinical Medicine

基　　金：国家自然科学基金资助项目(编号:81373192)。

摘　　要：目的分析2例早期前T淋巴母细胞白血病/淋巴瘤(early T-cell precursor acute lymphoblastic leukemia/lymphoma,ETP-ALL/LBL)患者的临床及实验室资料,结合文献复习,提高对该病的认识。方法回顾性分析我院诊治的2例ETP-ALL/LBL患者病例资料,并进行文献检索分析。结果2例患者均为中年,女性,以淋巴结肿大为主要表现起病。淋巴结活检病理诊断为T-淋巴母细胞淋巴瘤(T-LBL),流式细胞术免疫分型符合ETP-ALL/LBL(病例1为胸水标本,病例2为淋巴结标本)。病例1先后给予VDCLP方案及Hyper-CVAD方案化疗,病例2给予BFM-90方案化疗,2例患者化疗后均无效,因疾病进展死亡。结论ETP-ALL/LBL具有独特的免疫表型特点,病理诊断时注意加做早期标记和髓系抗体;ETP-ALL/LBL常规化疗效果较差,复发率高,预后差,强化疗及早期行造血干细胞移植可以改善预后。Objective The clinical and laboratory data of two patients with early T-cell precursor acute lymphoblastic leukemia/lymphoma(ETP-ALL/LBL)were analyzed.Combined with the review of previous literatures to provide a knowledge base for the clinical diagnosis and treatment.Methods The clinical data of 2 patients with ETP-ALL/LBL diagnosed and treated in our hospital were analyzed retrospectively,and a literature retrieval was performed for the progress analysis.Results The two patients were middle-aged and female,with lymph node enlargement as the main manifestation.Pathological diagnosis of lymph node biopsy was T-lymphoblastic lymphoma(T-LBL),and flow cytometry immunotyping result was consistent with ETP-ALL/LBL(case 1 was pleural effusion specimen,case 2 was lymph node specimen).Case 1 was treated with VDCLP regimen and Hyper-CVAD regimen successively,while case 2 was treated with BFM-90 regimen.Both patients failed after chemotherapy and died due to disease progressions.Conclusion ETP-ALL/LBL ETP-ALL/LBL has unique immunophenotypic characteristics.Early markers and myeloid antibodies should be added in the pathological diagnosis panel.ETP-ALL/LBL patients have a poor effect of conventional chemotherapy,high recurrence rate and poor prognosis.Intensive chemotherapy and early hematopoietic stem cell transplantation can improve the prognosis.

关 键 词：早期前T淋巴母细胞白血病/淋巴瘤 免疫表型 基因突变 预后 

分 类 号：R733.4[医药卫生—肿瘤]