EBV阴性侵袭性NK细胞白血病临床病理观察

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.

Clinicopathological features of Epstein-Barr virus negative aggressive NK cell leukemia

Objective To study the clinicopathological characteristics of Epstein-Barr virus negative aggressive NK cell leukemia (ANKL) and to improve our knowledge of the disease. Methods An unusual case of ANKL was analyzed in terms of clinical data, histological morphology, immunohistochemistry and EBER in situ hybridization, with a review of the relevant literatures for studying its clinicopathological characteristics and differential diagnosis. Results The patient of ANKL first presented with the hepatosplenomegly and cytopenia, later with a fever and lymphadenopathy. Tumor cells involved finally in spleen, liver, lymph nodes, bone marrow and blood. The neoplastic cells specifically expressed CD2,CD3 and CD56, but not cytotoxic molecules such as granzyme B. EBER was not detected in the samples. Clinical course of the case was relatively longer with a duration of 28 months as comparied with typical ANKL. Conclusion EBV negative ANKL shows a feature of relatively slow clinical progress, suggesting that it may be a special subtype of ANKL.