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+ CD4 -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.

abstractsObjective 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 wcrc 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 cases. Lymphoid nodules were present in 4 cases. Flow cytometery showed an immunophenotype of CD3 + CD4 - CD8 + CD56 - CD57 + of the tumor cells in 13 cases.Of the other 6 cases,the immunophenotypes included CD8- ( 1 case), CD56+ (2 cases) and CD57 ( 3 cases).Immunohistochemistry showed 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 rcccptor γ gene rearrangement by PCR was detected in 12 cases (12/17).Conclusions Hematopathologic features of most T-LGLL are distinct.Morphologic,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.