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.

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。