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目的分析和总结侵袭性NK细胞白血病(ANKL)的临床病理特点以及与EB病毒之间的关系,提高对该肿瘤的认识。方法对1例EB病毒阴性的ANKL特殊病例进行临床表现、组织形态、免疫组化以及原位杂交检测结果的分析,并结合文献讨论其临床病理特点及鉴别诊断。结果本例ANKL以白细胞、血小板减少和肝脾肿大为首发症状,继而出现腹水、发热和淋巴结肿大,并出现瘤细胞累及血液、骨髓及淋巴结,病程为2年4个月;病理形态学上,肿瘤细胞中等大小,轻度异型,在受累病变中弥漫浸润;免疫组化CD2、cCD3和CD56(+),CD5(-),细胞毒性分子(-),EB病毒(-)。结论EB病毒阴性的ANKL具有相对缓慢的临床进展特点,可能是一种特殊类型的ANKL白血病。
Objective To analyze and summarize the clinicopathological characteristics of invasive NK cell leukemia (ANKL) and its relationship with Epstein-Barr virus (EBV) and to improve the understanding of this tumor. Methods The clinical manifestations, histological features, immunohistochemistry and in situ hybridization results of 1 EBV negative ANKL were analyzed. The clinical and pathological features and differential diagnosis were also discussed in combination with the literature. Results In this case, leukopenia, thrombocytopenia and hepatosplenomegaly were the main symptoms of ANKL, followed by ascites, fever and lymph nodes, and the tumor cells involved blood, bone marrow and lymph nodes. The course of disease was 2 years and 4 months. Pathological morphology On the other hand, the tumor cells were moderately sized and mildly atypical, diffusing infiltrates in affected lesions. Immunohistochemical staining for CD2, cCD3 and CD56 (+), CD5 (-), cytotoxic molecules (-) and Epstein-Barr virus (-) Conclusion EB virus-negative ANKL has a relatively slow clinical features and may be a special type of ANKL.