T大颗粒淋巴细胞白血病伴纯红细胞再生障碍特征与治疗

摘要

Objective:To explore the clinical features and treatment of T-cell large granular lymphocytic leukemia(T-LGLL) associated with pure red cell aplasia(PRCA).Methods:Two cases of T-LGLL with PRCA were reported and the related articles were reviewed.Results:Two elder female patients had indolent process,mainly presenting with anemia.Clinic examination showed no lymphadenopathy and hepatosplenomegaly.The patients had no recurrent infections and rheumatoid arthritis.Peripheral blood smear showed predominance of mature lymphocytic cells,with most showing characteristic large granular lymphocytic morphology.Flowcytometric immunophenotypic analyses were done and peripheral lymphocytes showed abnormal immunophenotypes which were compatible with T-LGLL.Clonality could be proven on detecting gene rearrangement of T-cell recepter γchain.Bone marrow aspirate smears showed markedly decreased erythroid series.Conclusion:T-LGLL is a rare disease.The clinic characteristics of Asian patients were markedly different from Western patients.Especially,Asian patients had more anemia attributing to a high incidence of PRCA.The findings suggested that T-LGLL with PRCA may be prognostic of a good response to immunosuppression.%目的:探讨T-大颗粒淋巴细胞白血病(T-LGLL)伴纯红细胞再生障碍(PRCA)的临床特征与治疗.方法:报告2例T-LGLL合并PRCA并结合文献进行复习.结果:患者为老年女性,临床进展缓慢,主要表现为贫血,无淋巴结和肝脾肿大,无反复感染和类风湿性关节炎表现,血涂片以大颗粒淋巴细胞为主,免疫分型符合T-LGLL,TCRγ基因重排阳性,骨髓红系细胞明显减少.结论:T-LGLL为少见疾病,亚洲患者临床特征与西方患者有明显差异,尤其是合并PRCA的发生率高,对免疫抑制剂治疗反应良好.