大颗粒淋巴细胞白血病自然杀伤细胞型1例并文献复习

Large granular lymphocyte leukemia type natural killer cell one case and review of literature

目的:提高对自然杀伤(NK)细胞型大颗粒淋巴细胞白血病(NK-LGLL)的认识。方法:报告1例我院新近诊断的NK-LGLL的临床特征并结合文献进行复习讨论。结果:NK-LGLL为一少见疾病,临床以全身症状明显,肝、脾、淋巴结肿大多见。肿瘤细胞表现为CD3-、CD7+、CD16+、CD56+、CD4-、CD8-,提示NK细胞来源。该病临床进展迅速,预后差。结论:该病少见,需结合临床、组织学检测、免疫分型综合分析并加强与其他疾病的鉴别诊断。

Objective：To study the diagnose and treatment of large granular lymphocyte leukemia type natural killer cell （NK-LGLL）. Method：To report one case we have found recently and literature review and discussion. Result：NK-LGLL is a rare disease, its clinical characters are obviously general symptom, hepatomegaly, spleno- megaly, lymphadenectasis. The immunophenotype of cell is CD3^-,CD7^＋、 CD16^＋ 、CD56^＋、CD4^-、CD8^-、 All these indicate NK cell is the origin. This disease develop quickly and has bad prognosis. Conclusion： As this disease is rare, its diagnose should include clinical manifestation, histochemical staining, immunophenotype and differenti- al diagnosis with other disease.