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再生障碍性贫血免疫抑制治疗后并发的爱泼斯坦-巴尔病毒相关自然杀伤/T细胞淋巴瘤:一例报告并文献复习

Concurrent Epstein-Barr virus associated NK/T cell lymphoma after immunosuppressive therapy for aplastic anemia: report of a case and review of literature.

作者信息

Yin Guangli, Ni Ying, Xiao Zhengrui, He Guangsheng, Miao Kourong

机构信息

Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital Nanjing 210029, China.

出版信息

Int J Clin Exp Pathol. 2015 Jun 1;8(6):7588-93. eCollection 2015.

Abstract

Aplastic anemia (AA) patients with prolonged immunosuppression have a risk of development of lymphoproliferative disorders (LPDs), especially combined with Epstein-Barr virus (EBV) infection. However, development of nature killer/T (NK/T) cell lymphoma, in a nontransplantation setting, has not been documented for AA patients with immunosuppressive therapy (IST). Herein, we described a middle-aged man, Han ethnic, who presented with swelled parotid gland after a long history of IST for AA. Fever, night sweating, weight loss had not been found. Increased heterotypic lymphocytes had been detected in the left side of parotid gland demonstrated as cCD3(+), CD56(+), GranB(+), TIA-1(+), MUM-1(+), KI-67 (50%-75%)(++), Bcl-6(-), MPO(-) by immunohistochemistry, and in-situ hybridization (ISH) indicated EBER positive. Chromosome analysis by R banding method revealed 46, XY [20]. NK/T cell lymphoma concurrent with aplastic anemia was diagnosed and a mild chemotherapy regimen including vincristine, prednisone, L-asparaginase was administered. The parotid mass was gradually regressed after the first cycle of chemotherapy. The patient discharged from the hospital voluntarily and lost the follow-up.

摘要

长期免疫抑制的再生障碍性贫血(AA)患者有发生淋巴增殖性疾病(LPDs)的风险,尤其是合并爱泼斯坦-巴尔病毒(EBV)感染时。然而,在非移植情况下,接受免疫抑制治疗(IST)的AA患者发生自然杀伤/T(NK/T)细胞淋巴瘤的情况尚未见报道。在此,我们描述了一名汉族中年男性,他在长期接受AA的IST治疗后出现腮腺肿大。未发现发热、盗汗、体重减轻。腮腺左侧检测到异型淋巴细胞增多,免疫组化显示为cCD3(+)、CD56(+)、颗粒酶B(+)、TIA-1(+)、MUM-1(+)、KI-67(50%-75%)(++)、Bcl-6(-)、MPO(-),原位杂交(ISH)显示EBER阳性。采用R带法进行染色体分析显示为46, XY [20]。诊断为NK/T细胞淋巴瘤并发再生障碍性贫血,并给予包括长春新碱、泼尼松、L-天冬酰胺酶的轻度化疗方案。化疗第一个周期后腮腺肿块逐渐消退。患者自动出院,失去随访。

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