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目的探讨侵袭性自然杀伤细胞白血病(ANKL)的临床特点、诊断与鉴别诊断要点及诊治关键。方法对1例ANKL合并噬血细胞综合征及弥散性血管内凝血患者的临床资料结合文献复习进行分析。结果患者高热、脾肿大、进行性血细胞减少、凝血异常、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、CD38阳性,CD3、CD7、CD8和CD117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,该病常早期侵袭性进展,治疗效果欠佳,预后极差。
Objective To investigate the clinical features of invasive natural killer cell leukemia (ANKL), the key points of diagnosis and differential diagnosis and diagnosis. Methods One case of ANKL with hemophagocytic syndrome and disseminated intravascular coagulation in patients with clinical data combined with literature review for analysis. Results The leukemia cell immunophenotype was positive for CD2, CD56, CD38, negative for CD3, CD7, CD8 and CD117 in patients with hyperthermia, splenomegaly, progressive cytopenia, abnormal coagulation, abnormal bone marrow cells with phagocytic cells, Rapid clinical progress. Conclusion ANKL is a rare disease. In addition to the clinical manifestations, it is necessary to combine the immunophenotypes of leukemia cells with the diagnosis and to pay attention to the identification of other related diseases. The disease is often characterized by early aggressive progress, poor treatment outcome and poor prognosis.