摘要目的 探讨侵袭性NK细胞白血病(ANKL)的临床病理学特点.方法 回顾性分析10例ANKL患者的临床病理档案资料,全部病例均行全血细胞计数以及外周血涂片、骨髓穿刺与骨髓活检标本的形态学观察.用流式细胞学(FCM)及免疫组织化学(EliVision法)进行免疫表型分析.聚合酶链反应(PCR)法检测T细胞受体(TCR)γ基因重排.结果 10例患者中,最常见的血液学异常为贫血(7例)与血小板减少(9例).6例外周血涂片可见大颗粒淋巴细胞.骨髓穿刺涂片示8例淋巴细胞比例增高(＞20.0％).6例可见大颗粒淋巴细胞.骨髓活检切片示轻度浸润5例,中度浸润3例,重度浸润2例.骨髓切片中8例为间质型浸润,2例呈弥漫型浸润,4例可见噬血现象(吞噬成熟红细胞).免疫表型方面,FCM检测示全部病例为CD2+sCD3- CD4- CD56+CD57-.9例CD7、5例CD16、4例CD8和1例CD5阳性.8例行免疫组织化学相关抗原检测:cCD3 4例、CD566例、T细胞内抗原1(TIA-1)6例、颗粒酶B4例和穿孔素2例阳性.10例TCRγ基因重排检测均为胚系构型.结论 ANKL是一种NK细胞来源的高度侵袭性淋巴组织肿瘤,需进行全面的外周血与骨髓的形态学、免疫表型以及分子遗传学检测才能确诊,需注意与多种NK细胞与T细胞淋巴瘤鉴别.

abstractsObjective To study the clinicopathologic features of aggressive natural killer cell leukemia (ANKL).Methods The clinical and pathologic features were analyzed in 10 patients with ANKL.The complete blood count,peripheral blood smears,bone marrow aspirates and bone marrow biopsies were studied. Immunophenotypic analysis was carried out by flow cytometry and immunohistochemistry.T-cell receptor (TCR) γ gene rearrangement was studied by PCR method.Results The most frequent hematologic abnormalities observed were anemia (7 cases ) and thrombocytopenia (9 cases).Large granular lymphocytes were found on peripheral blood smears of 6 patients.In bone marrow aspirates,lymphocytosis ( ＞ 20.0％ ) was demonstrated in 8 cases and large granular lymphocytes in 6 cases.Bone marrow biopsies revealed various degrees of neoplastic infiltration,as follows:mild (5 cases),moderate (3 cases) and severe (2 cases).The neoplastic cells were mainly interstitial in distribution in 8 cases and diffuse in 2 cases.Hemophagocytosis was observed in 4 cases.Flow cytometry showedCD2+ sCD3- CD4- CD56+ CD57- in all cases,CD7 + in9 cases,CD16+ in 5 cases,CD8 + in 4 cases and CD5 + in 1 case.lmmunohistochemistry performed in 8 cases showed the following results:cCD3 + in 4 cases,CD56 + in 6 cases,TIA-1 + in 6 cases,granzyme B + in 4 cases and perforin + in 2 cases.PCR study revealed germline TCRγ gene configuration in all cases.Conclusions ANKL is a highly aggressive NK cell-derived lymphoid neoplasm. Comprehensive morphologic,immunophenotypic and molecular analysis are essential in arriving at a correct diagnosis.ANKL needs to be distinguished from other types of NK-cell and T-cell lymphomas.