T大颗粒淋巴细胞白血病的血液病理学特点  

Henmtopathologic features of T-cell large granular lymphocytic leukemia

作　　者：刘恩彬[1] 陈辉树[1] 张培红[1] 李占琦[1] 孙琦[1] 杨晴英[1] 方立环[1] 孙福军[1] 

机构地区：[1]中国医学科学院北京协和医学院血液病医院病理科,天津300020

出　　处：《中华病理学杂志》2012年第4期229-233,共5页Chinese Journal of Pathology

摘　　要：目的探讨T大颗粒淋巴细胞白血病（T—LGLL）的血液病理学特点。方法回顾性分析2005年11月至2010年4月收集的19例T—LGLL患者的临床资料、骨髓形态、免疫表型及分子遗传学情况。结果19例患者中最常见的血液学异常为贫血（16例）与中性粒细胞减少（17例）。17例外周血涂片可见大颗粒淋巴细胞。骨髓涂片示17例淋巴细胞比例增高（〉0．2），15例可见大颗粒淋巴细胞。骨髓活检切片示16例淋巴细胞增多，其中12例为轻至中度增多。骨髓切片中淋巴细胞均为间质型分布，8例可见血窦内分布，4例可见淋巴细胞结节。流式细胞学示13例CD3＋CIM—CD8＋CD56-CD57＋。6例免疫标记不典型，为CD8—1例、CD56＋2例、CD57—3例。免疫组织化学示CD3（10／10）、CD57（3／3）、CD8（6／7）、T细胞胞内抗原-1（TIA-1，6／7）、颗粒酶B（4／7）、穿孔素（1／7）阳性，CD4（4／4）、CD56（9／9）阴性。T细胞受体（TCR）γ基因重排检测12例阳性（12／17）。结论大多数T—LGLL具有典型的血液病理学特点。外周血与骨髓的形态学、免疫表型及分子遗传学检测是T—LGLL诊断与鉴别诊断中必不可少的内容，三者互为补充。Objective To explore the hematopathologic features of T-cell large granular lymphocytic leukemia （T-LGLL） . Methods A retrospective analysis of the clinical presentation, bone marrow morphology, immunophenotyping and T-cell receptor gene rearrangement status were performed in 19 patients with T-LGLL Results Of 19 patients, the most frequent hematological abnormalities were anemia and neutropenia （16/19 and 17/19 patients, respectively）. Large granular lymphocytes （LGLs） were observed in 17 of 19 peripheral blood smears and 15 of 19 bone marrow aspirate specimens. Lymphocytosis （ 〉0. 2） was present in 17 of 19 patients in their bone marrow aspirate specimens. Bone marrow biopsy specimens revealed lymphocytosis in 16 cases, with a mild to moderate increase of lymphocytes observed in 12 cases （12/16）. The pattern of lymphoid distribution was interstitial in bone marrow sections. Intravascular distribution was seen in 8 eases. Lymphoid nodules were present in 4 cases. Flow cytometery showed an immunophenotype of CD3＋ CD4 - CD8 ＋ CD56 - CD57 ＋ of the tumor cells in 13 eases. Of the other 6 cases, the immunophenotypes included CD8- （1 case）, CD56＋ （2 cases ） and CD57- （3 cases） . Immunohistochemistryshowed CD3 ＋ （10/10）, CD57 ＋ （3/3）, CD8 ＋ （6/7）,TIA-1 ＋ （6/7）, granzyme B ＋ （4/7）, perforin ＋ （1/7）, CD4 - （4/4） and CD56 - （9/9）. Clonal T-cell receptor γ gene rearrangement by PCR was detected in 12 cases （12/17）. Conclusions Hematopathologic features of most T-LGLL are distinct. Morphologie, immunophenotypic and molecular analysis of both peripheral blood and bone marrow specimens are essential and complementary in the diagnosis and differential diagnosis of T-LGLL.

关 键 词：白血病 大颗粒淋巴细胞 病理学 临床 骨髓检查 

分 类 号：R733.7[医药卫生—肿瘤]