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目的提高对侵袭性NK细胞白血病(ANKL)的认识。方法报告近期诊断的1例ANKL患者,并进行文献学习。结果该病发病率很低,亚洲人群高发,EB病毒感染与其相关,该病呈高侵袭性,临床病程进展快速,病变累及多脏器,以腰酸、腹痛、恶心为首发表现,预后差,极其罕见。结论ANKL是一种少见的白血病,主要与结外NK/T淋巴瘤和惰性NK细胞淋巴细胞增生性疾病相鉴别,应及时做免疫表型(特征为CD2+sCD3-CD56+)、基因重排(TCR重排阴性)及病理学检查,以明确诊断。
Objective To improve the understanding of aggressive NK cell leukemia (ANKL). Methods One recent report of ANKL was reported and documented. Results The incidence of this disease was very low. The population of Asia was high. EB virus infection was related to it. The disease was highly aggressive. The clinical course progressed rapidly. The lesions were complicated by multiple organs. Backache, abdominal pain and nausea were the first manifestations. The prognosis was poor and extremely poor rare. Conclusion ANKL is a rare leukemia, mainly associated with extranodal NK / T lymphoma and idiopathic NK cell lymphoproliferative diseases, and should be promptly immunophenotype (characterized by CD2 + sCD3-CD56 +), gene rearrangement TCR rearrangement negative) and pathological examination to confirm the diagnosis.