侵袭性NK细胞白血病3例并文献复习

Aggressive NK cell leukemia:a report of three cases and review of literature

目的讨论侵袭性NK细胞白血病(ANKL)的临床表现、病理学特点。方法收集蚌埠医学院第一附属医院近年来收治的3例ANKL患者的临床与病理学资料,分析其临床表现与病理学特点,进行骨髓穿刺及骨髓活检标本观察形态学,用流式细胞学及免疫组化对免疫表型进行分析,并文献复习。结果3例患者为1名男性和2名女性,均以发热、脾大及全血细胞减少为主要临床表现,病理形态学上累及淋巴结时常表现为肿瘤细胞形态相对一致,细胞体积中等大小,核呈圆形或不规则,可伴组织坏死,核分裂象易查见。免疫组化表型:CD20(-)、膜CD3(+)、CD56(++)、Ki-67(+,70%),原位杂交EBER阳性率表达。结论ANKL进展迅速,预后差,常规化疗对于该病基本无效,细胞移植治疗对患者生存预后有影响。

Objective To discuss the clinical and pathological features of aggressive NK cell leukemia(ANKL).Methods The clinical and pathological data of 3 patients with ANKL admitted to our hospital in recent years were collected,and the clinical and pathological features were analyzed.Bone marrow puncture and bone marrow biopsy specimens were used to observe the morphology,and the immunophenotype was analyzed by flow cytometry and immunohistochemistry.Results Three patients included 1 men and two women,and presented with fever,spleen enlargement and complete blood decrease as the main clinical manifestations.The pathological morphology on the involvement of lymph nodes was often characterized by tumor cells forming relatively consistent cell infiltration of medium size and round or irregular neclei,which were associated with necrosis,and obvious mitosis.Immunohistochemical phenotypes showed that the tumor cells were negative for CD20 and positive for CD3 and CD56;Ki-67 index was>60%,and EBER positive rate was about 50%by in situ hybridization.Conclusion ANKL progresses rapidly and the prognosis is poor.Conventional chemotherapy is basically ineffective for ANKL,and cell transplantation has an impact on the survival prognosis of patients.