侵袭性NK细胞白血病1例并文献复习

Aggressive NK cell leukemia:A case report and literature review

目的:提高对侵袭性NK细胞白血病(ANKL)的认识。方法:报告1例ANKL患者并结合文献进行复习。结果:患者高热、肝脾肿大、进行性血细胞减少、外周血大颗粒淋巴细胞异常增多、骨髓中异常细胞增多并伴吞噬血细胞现象等,白血病细胞免疫表型为CD2、CD56阳性,CD3、CD4、CD8和CD57阴性,临床进展迅速。结论:ANKL为一种少见疾病,除临床表现外,诊断时需结合白血病细胞的免疫表型并需注意与其他相关疾病的鉴别,该病常呈侵袭性进展,治疗效果欠佳,预后极差。

Objective ：To improve the recognition of aggressive NK cell leukemia （ANKL）. Methods： A case of ANKL was discussed with the review of related articles. Results： The patient had following characters： high fever, hepatosplenomegaly, pancytopenia, increased large granular lymphocyte （LGL） in peripheral blood and abnormal cells in marrow. The hemophagocytes were observed in marrow. The immunophenotypes of leukemic cells were CD2^＋ , CD56^＋ , CD3^-, CD4^-, CD8^-, CD57^-. This disease developed very quickly. Conclusion： As ANKL is rare, its diagnosis should include clinical presentation,immunophenotype and excluse other diseases such as large granular lymphocytosis. This disease is aggressive, the treatment is usually ineffective and the prognosis is very poor.