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EBV-positive large B-cell lymphoma with an unusual intravascular presentation and associated haemophagocytic syndrome in an HIV-positive patient: report of a case expanding the spectrum of EBV-positive immunodeficiency-associated lymphoproliferative disorders
Virchows Archiv ( IF 3.4 ) Pub Date : 2021-06-19 , DOI: 10.1007/s00428-021-03142-1
Luis Veloza 1 , Chun-Yi Tsai 1 , Bettina Bisig 1 , Olivier Pantet 2 , Lorenzo Alberio 3 , Christine Sempoux 1 , Matthias Cavassini 4 , Laurence de Leval 1
Affiliation  

Intravascular large B-cell lymphoma is a rare and aggressive EBV-negative large B-cell lymphoma with a dismal outcome. Here, we describe the case of a 76-year-old HIV-positive patient with an acute presentation of systemic symptoms and rapidly fatal outcome. Autopsy revealed a disseminated large B-cell lymphoma with an intravascular distribution involving the liver, lymph nodes, spleen, and bone marrow and associated to fibrin thrombi in hepatic capillary haemangiomas. The neoplastic B cells (CD79a + / − , CD20 + / − , CD30 + , MUM1 + , PD-L1 +) showed a Hodgkin and Reed-Sternberg-like morphology and were EBV-positive with a latency type II (LMP1 + , EBNA2-). Haemophagocytosis was documented in the bone marrow and lymph nodes. This case illustrates the diagnostic challenges of large B-cell lymphoma with intravascular presentation. We found only five other cases of EBV-positive large B-cell lymphoma with an intravascular presentation in the literature, three of which had an underlying immunodeficiency adding to the broad spectrum of EBV-associated lymphoma in the setting of immunosuppression.



中文翻译:

在 HIV 阳性患者中出现异常血管内表现和相关噬血细胞综合征的 EBV 阳性大 B 细胞淋巴瘤:一例扩大 EBV 阳性免疫缺陷相关淋巴组织增生性疾病谱的病例报告

血管内大 B 细胞淋巴瘤是一种罕见的侵袭性 EBV 阴性大 B 细胞淋巴瘤,预后不佳。在这里,我们描述了一名 76 岁 HIV 阳性患者的病例,该患者具有急性全身症状和迅速致命的结果。尸检显示播散性大 B 细胞淋巴瘤,其血管内分布涉及肝脏、淋巴结、脾脏和骨髓,并与肝毛细血管瘤中的纤维蛋白血栓有关。肿瘤性 B 细胞 (CD79a + / - , CD20 + / - , CD30 + , MUM1 + , PD-L1 +) 表现出霍奇金和 Reed-Sternberg 样形态,EBV 阳性,潜伏期 II 型 (LMP1 + , EBNA2-)。在骨髓和淋巴结中记录到噬血作用。该病例说明了血管内表现的大 B 细胞淋巴瘤的诊断挑战。

更新日期:2021-06-19
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