Division of Haematology, Department of Pathology, Faculty of Health Sciences, University of Cape Town and National Health Laboratory Service, Groote Schuur Hospital, Cape Town, South Africa.
Division of Anatomical Pathology, Department of Pathology, Faculty of Health Sciences, University of Cape Town and National Health Laboratory Service, Groote Schuur Hospital, Cape Town, South Africa.
Int J Lab Hematol. 2022 Sep;44 Suppl 1(Suppl 1):54-63. doi: 10.1111/ijlh.13863.
Plasmablastic lymphoma (PBL) is a highly aggressive B cell non-Hodgkin lymphoma frequently associated with immunosuppression, particularly human immunodeficiency virus (HIV) infection. Although PBL is rare globally, South Africa has a high burden of HIV infection leading to a higher incidence of PBL in the region. Laboratory features in PBL may overlap with plasmablastic myeloma and other large B cell lymphomas with plasmablastic or immunoblastic morphology leading to diagnostic dilemmas. There are, however, pertinent distinguishing laboratory features in PBL such as a plasma cell immunophenotype with MYC overexpression, expression of Epstein-Barr virus-encoded small RNAs and lack of anaplastic lymphoma kinase (ALK) expression. This review aims to provide a summary of current knowledge in PBL, focusing on the epidemiology, pathophysiology, laboratory diagnosis and clinical management.
浆母细胞淋巴瘤（PBL）是一种高度侵袭性的 B 细胞非霍奇金淋巴瘤，常与免疫抑制有关，特别是人类免疫缺陷病毒（HIV）感染。尽管 PBL 在全球范围内较为罕见，但南非 HIV 感染负担沉重，导致该地区 PBL 的发病率较高。PBL 的实验室特征可能与浆母细胞骨髓瘤和其他具有浆母细胞或免疫母细胞形态的大 B 细胞淋巴瘤相重叠，导致诊断难题。然而，PBL 存在一些相关的鉴别实验室特征，如浆细胞免疫表型伴 MYC 过表达、表达 Epstein-Barr 病毒编码的小 RNA 以及缺乏间变性淋巴瘤激酶（ALK）表达。本文旨在综述 PBL 的现有知识，重点介绍其流行病学、发病机制、实验室诊断和临床管理。
