侵袭性NK细胞白血病1例报告并文献复习

Agressive NK-Cell Leukemia:A Case Report and Literature Review

目的:提高对侵袭性NK细胞白血病的认识。方法:对1例侵袭性NK细胞(ANKL)白血病患者的临床资料进行分析,并复习国内外相关文献。结果:该病好发于中青年,病程短,进展快,平均生存期2个月;ANKL患者常表现为发热及肝、脾、淋巴结肿大;外周血、骨髓涂片大颗粒淋巴细胞增多;免疫表型CD 2(+),表面CD 3(-),胞质CD 3(+),CD 56(+),CD 57(-);TCR基因重排均为阴性;有EB病毒感染的证据;常见染色体异常;排除其他大颗粒淋巴细胞白血病。结论:ANKL是一种罕见病,恶性度高,预后极差。

Objective：To improve the recognization of aggressive NK-cell leukemia（ANKL）. Method：The clinical datum of one case of aggressive NK-cell leukemia was analyzed and the related literature was reviewed. Results ：The disease was commonly seen in young and middle-aged people. The disease had an aggressive clinical course and the median overall survival time of patients was 2 months. The paitents often present with fever, hepatosplenomegly and lymphadenopathy;increasing large granular lymphocytes could be finded in bone marrow and blood ;immunophenotype：CD2 （＋） ,suffaceCD3（-） ,cytoplasmCD3 （＋） ,CDS6（＋） ,CD57（-） ;TCR genes rearrangement negative; EB virus antibody usually positive;chromosomal abnormality usuallly exist;exclusion of other disease with large granular lymphocyte. Conclusion ：ANKL is extremely rare and high grade malignancy,it carries a very poor prognosis.