Gray Austin L, Ding Daniel, Ncube Ziphezinhle, Mukherjee Kaushik, Kerstetter Justin, Liu Yan, Wang Jun
Department of Pathology and Laboratory Medicine, Loma Linda University Medical Center, Loma Linda, California, USA.
Division of Acute Care Surgery, Loma Linda University Medical Center, Loma Linda, California, USA.
J Gastrointest Oncol. 2022 Jun;13(3):1467-1472. doi: 10.21037/jgo-21-779.
Plasmablastic lymphoma (PBL) is a rare, highly aggressive lymphoma of plasma cell differentiation. It commonly presents as an Epstein-Barr virus (EBV)+ oral lesion in an immunodeficient patient, predominately human immunodeficiency virus (HIV)+ patients. These aggressive lesions often demonstrate an immunoblastic or plasmablastic morphology with a typical immunohistochemical profile. The current case is unique due to the location at presentation, immunohistochemical features, and unknown presence of HIV infection in a young adult male.
We present an unexpected case of PBL found in a rare extra-oral location in a young adult male with undiagnosed HIV infection presenting as a perianal hemorrhoid mass/abscess. Swift treatment for HIV and the PBL resulted in complete remission and markedly improved CD4 counts.
This case highlights the importance of testing for HIV along with acquiring a thorough social/clinical history when a PBL is encountered. Although the overall prognosis of PBL is dismal with a median survival of about 6-11 months, a timely accurate diagnosis and prompt chemotherapy with an appropriate regimen along with antiretroviral therapy (ART) may still achieve a successful outcome with a relatively reasonable long-term remission like in our reported case.
浆母细胞淋巴瘤（PBL）是一种罕见的、具有高度侵袭性的浆细胞分化淋巴瘤。它通常在免疫缺陷患者中表现为 Epstein-Barr 病毒（EBV）阳性的口腔病变，主要是人类免疫缺陷病毒（HIV）阳性患者。这些侵袭性病变通常表现为免疫母细胞或浆母细胞形态，并具有典型的免疫组化特征。本病例因其发病部位、免疫组化特征以及一名年轻成年男性患者中未知的 HIV 感染情况而独特。
我们报告了一例意外的 PBL 病例，该病例发生在一名未诊断出 HIV 感染的年轻成年男性的罕见口腔外部位，表现为肛周痔块/脓肿。对 HIV 和 PBL 的迅速治疗导致完全缓解，并显著提高了 CD4 细胞计数。
本病例强调了在遇到 PBL 时检测 HIV 以及获取全面的社会/临床病史的重要性。尽管 PBL 的总体预后不佳，中位生存期约为 6 - 11 个月，但及时准确的诊断、采用适当方案的及时化疗以及抗逆转录病毒疗法（ART）仍可能取得成功结果，如我们报告的病例那样实现相对合理的长期缓解。
