大颗粒淋巴细胞白血病的诊断及治疗  

The diagnosis and treatment of the large granular lymphocytic leukemia

作　　者：周剑峰[1] 孟凡凯[1] 

机构地区：[1]华中科技大学同济医学院附属同济医院血液科,武汉430030

出　　处：《中国实用内科杂志》2015年第2期107-109,共3页Chinese Journal of Practical Internal Medicine

基　　金：湖北省自然科学基金项目(2013CFB098);武汉市科技局应用基础研究计划项目(2013062301010801)

摘　　要：大颗粒淋巴细胞白血病(LGLL)是一起源于CD3+CTL和CD3-NK细胞的克隆性增殖性疾病,以肿瘤细胞胞浆中含有嗜苯胺蓝颗粒为特征。该病临床表现有惰性和侵袭性之分。其诊断需结合患者临床表现以及大颗粒淋巴细胞的形态学、免疫表型、分子遗传学等指标来确立。对于惰性的无临床症状的LGLL患者只需进行随访观察,无须治疗,对于有症状者推荐行免疫抑制治疗。对于侵袭性LGLL患者,急性淋巴细胞白血病样化疗加异基因造血干细胞移植可能是有效治疗。Large granular lymphocytic leukemia（LGLL）is a clonal lymphoproliferative disease that arises from CD3＋CTL or CD3 NK cell lineages.The malignant large granular lymphocytes（LGLs） are characterized by the typical azurophilic granules in the cytoplasm. Clinical processes of LGLL manifest as the indolent or aggressive subtype.q-he diagnosis of LGLL is based on the combination of the clinical presentation and the morphology,immunophenotype and the molecular genetics of the LGLs.Clinically asymptomatic patients need no treatment,but only watchful follow-up.For the asymptomatic patients of indolent LGLL,immunosupressive therapy is usually recommended as the first line treatment.For the aggressive LGLL patients,the treatment of acute lymphoblastic leukemia type chemotherapy combined with allogenic hematopoietic stem cell transplantation may be effective.

关 键 词：大颗粒淋巴细胞白血病 免疫抑制治疗 造血干细胞移植 

分 类 号：R5[医药卫生—内科学]