大颗粒淋巴细胞白血病三例并文献复习

Large granular lymphocyte leukemia： three cases report and review of the literature

目的 探讨大颗粒淋巴细胞白血病（LGLL）的临床特征及诊疗方法.方法 回顾性分析3例LGLL患者的临床资料并复习相关文献.结果 3例患者均以淋巴细胞增多起病,病情进展缓慢,其中2例为T-LGLL,免疫表型特征为CD3＋ CD4-CD8＋ CD56-CD57＋,1例为NK-LGLL,免疫表型特征为CD3-CD4-CD8-CD56＋ CD57-.2例患者暂时不需要临床干预,1例患者因粒细胞缺乏及反复感染给予环孢素治疗.结论 LGLL是一组异质性疾病,其临床特征及预后各不相同.流式细胞术免疫表型分析及TCRVβ基因谱型分析、TCR基因重排等检测有利于明确诊断.

Objective To investigate the clinical characteristics and methods of diagnosis and treatment of granular lymphocytic leukemia （LGLL）.Methods Clinical data of 3 patients with LGLL were retrospectively analyzed and relevant literature was reviewed.Results 3 patients were all onset with lymphocytosis,whose conditions progressed slowly.The diagnosis of 2 patients was T-LGLL with immunological characteristics of CD3＋ CD4 CD8＋ CD56-CD57＋.The other patient＆#39; s diagnosis was NK-LGLL,whose immunological characteristic was CD3-CD4-CD8-CD56＋ CD57-.Two of them didn＆#39; t need any treatment.One of them was treated with cyclosporine because of agranulocytosis and recurrent infection.Conclusions LGLL is a group of heterogeneous diseases,which clinical characteristic and prognosis are different.Flow cytometric immunopheotype,TCR Vβ analysis and TCR gene rearrangement are helpful to diagnosis.