Willemsen Mathijs, Dielis Arne W J H, Samarska Iryna V, Koster Ad, van Marion Arienne M
Department of Internal Medicine, VieCuri Medical Centre, 5912 BL Venlo, Netherlands.
Department of Pathology, VieCuri Medical Centre, 5912 BL Venlo, Netherlands.
Case Rep Hematol. 2017;2017:1279525. doi: 10.1155/2017/1279525. Epub 2017 Apr 12.
Angioimmunoblastic T-cell lymphoma is a rare non-Hodgkin lymphoma with dismal prognosis. The median age of presentation ranges from 62 to 69 years with generalized lymphadenopathy, B symptoms, and hepatosplenomegaly as the most prevalent symptoms. The combination of B-cell and T-cell proliferations is common in AITL and the B-cell component may resemble Reed-Sternberg-like B-cells. Epstein-Barr virus is estimated to be present in 80-95% of AITL biopsies. Only a handful of EBV-negative AITL cases with EBV-negative RS-like B-cells have been reported over the last decade. We present a rare case of EBV-negative AITL with chylous ascites and chylothorax. Microscopic and immunohistochemical analysis revealed the presence of EBV-negative Reed-Sternberg-like B-cells in the tumor.
血管免疫母细胞性T细胞淋巴瘤是一种罕见的非霍奇金淋巴瘤，预后较差。发病的中位年龄在62至69岁之间，最常见的症状为全身淋巴结肿大、B症状以及肝脾肿大。B细胞和T细胞增殖同时出现的情况在血管免疫母细胞性T细胞淋巴瘤中很常见，且B细胞成分可能类似于里德-施特恩贝格样B细胞。据估计，80%至95%的血管免疫母细胞性T细胞淋巴瘤活检标本中存在爱泼斯坦-巴尔病毒。在过去十年中，仅有少数几例报告了伴有EBV阴性的里德-施特恩贝格样B细胞的EBV阴性血管免疫母细胞性T细胞淋巴瘤病例。我们报告了一例罕见的伴有乳糜性腹水和乳糜胸的EBV阴性血管免疫母细胞性T细胞淋巴瘤病例。显微镜检查和免疫组织化学分析显示肿瘤中存在EBV阴性的里德-施特恩贝格样B细胞。
