Zhou Yi, Rosenblum Marc K, Dogan Ahmet, Jungbluth Achim A, Chiu April
Department of Pathology, Loyola University Medical Center, Rm 2222, Building 110, 2160 S 1st Ave, Maywood, IL 60153, USA.
Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY 10065, USA.
J Hematop. 2015 Dec;8(4):235-241. doi: 10.1007/s12308-015-0241-8. Epub 2015 Mar 18.
Epstein-Barr virus (EBV)-associated B cell lymphoproliferative disorders may be seen in patients with angioimmunoblastic T cell lymphoma (AITL). Although both nodal and extranodal sites of involvement have been described, central nervous system involvement by B cell lymphoma following AITL has not previously been documented. We report a first example of such unusual presentation, in which an 80-year-old man developed diffuse large B cell lymphoma (DLBCL) in the cerebellum 4 months after the initial diagnosis of AITL. EBV-encoded RNAs were detected in the DLBCL, suggesting that EBV played a pivotal role in the pathogenesis of high-grade histologic progression of AITL. The patient survived less than 9 months after his initial diagnosis of AITL. We believe that this case expands the spectrum of extranodal manifestation of EBV-positive B cell lymphoma associated with AITL and illustrates the importance of recognition of this association when encountering unusual central nervous system lesions in patients with known AITL.
血管免疫母细胞性T细胞淋巴瘤(AITL)患者可能出现与爱泼斯坦-巴尔病毒(EBV)相关的B细胞淋巴增殖性疾病。尽管已有关于淋巴结和结外受累部位的描述,但此前尚无AITL后B细胞淋巴瘤累及中枢神经系统的记录。我们报告了这样一例罕见的病例,一名80岁男性在最初诊断为AITL后4个月,小脑出现弥漫性大B细胞淋巴瘤(DLBCL)。在DLBCL中检测到EBV编码的RNA,提示EBV在AITL高级别组织学进展的发病机制中起关键作用。该患者在最初诊断为AITL后存活不到9个月。我们认为,该病例扩展了与AITL相关的EBV阳性B细胞淋巴瘤的结外表现谱,并说明了在已知AITL患者中遇到不寻常的中枢神经系统病变时认识这种关联的重要性。