Bishnu S, Banerjee S, Bandyopadhyay D, Samui S, Bhattacharya S, Bose D
Department of Medicine, Medical College, Kolkata, West Bengal, India.
Indian J Cancer. 2015 Oct-Dec;52(4):563-7. doi: 10.4103/0019-509X.178437.
Plasmablastic lymphoma (PBL), a rare non-Hodgkin's lymphoma (NHL) variant specifically associated with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS), expresses well-differentiated plasma cell markers like CD138, bright CD38, and MUM1; but not conventional B-cell markers. It occurs at unusual sites like oral cavity and orbit, and has poor survival rates.
This study serves as a review of a clinical experience with six HIV patients with PBL and observes the spectrum of clinical presentations, histopathologies, and 1-year outcomes in PBL patients.
This review of six PBL patients was conducted at a tertiary care hospital in eastern India using relevant radiological, histopathogical, and immunohistological studies.
Incidence of PBL among HIV patients was 0.58% (6/1,028). Mean CD4 count at presentation was 125.5 ± 71.1 cells/μL. Sites of involvement included pleura, lung parenchyma, suprarenal gland, pelvic cavity, and retroorbital space (one each). Immunohistopathology of biopsied sample in each patient revealed PBL (positive plasma cell markers MUM-1/IRF4, CD38, and CD138/syndecan; and negative of B-cell markers CD3, CD20, and CD30). Three (60%) were positive for Epstein Barr virus (EBV) immunoglobulin G (IgG). Five surviving patients received CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) regimen and attained partial remission (PR) after six cycles. Subsequently, three patients were started on EPOCH (etoposide, cyclophosphamide, doxorubicin, vincristine, prednisone) therapy; two attained near total regression after 6 months (four cycles). Overall, four patients remained alive with good quality of life at the end of 1 year of follow-up.
PBL in HIV occurs at unusual sites with varying aggressivity. This study is too small to comment on the long-term outcomes of PBL in HIV; however, coadministration of antiretroviral therapy (ART) with standard chemotherapy may improve survival.
浆母细胞淋巴瘤（PBL）是一种罕见的非霍奇金淋巴瘤（NHL）变体，特别与人类免疫缺陷病毒/获得性免疫缺陷综合征（HIV/AIDS）相关，表达分化良好的浆细胞标志物，如CD138、明亮的CD38和MUM1；但不表达传统B细胞标志物。它发生在口腔和眼眶等不寻常部位，生存率较低。
本研究回顾了6例HIV相关PBL患者的临床经验，观察PBL患者的临床表现、组织病理学及1年预后情况。
在印度东部一家三级医院，通过相关影像学、组织病理学和免疫组织化学研究，对6例PBL患者进行回顾性分析。
HIV患者中PBL的发病率为0.58%（6/1028）。初诊时平均CD4细胞计数为125.5±71.1个/μL。受累部位包括胸膜、肺实质、肾上腺、盆腔和眶后间隙（各1例）。每位患者活检样本的免疫组织病理学检查均显示为PBL（浆细胞标志物MUM-1/IRF4、CD38和CD138/多配体蛋白聚糖呈阳性；B细胞标志物CD3、CD20和CD30呈阴性）。3例（60%）患者的爱泼斯坦-巴尔病毒（EBV）免疫球蛋白G（IgG）呈阳性。5例存活患者接受了CHOP（环磷酰胺、阿霉素、长春新碱、泼尼松）方案治疗，6个周期后达到部分缓解（PR）。随后，3例患者开始接受EPOCH（依托泊苷、环磷酰胺、阿霉素、长春新碱、泼尼松）治疗；2例患者在6个月（4个周期）后接近完全缓解。总体而言，随访1年末，4例患者存活，生活质量良好。
HIV相关PBL发生在不寻常部位，侵袭性各异。本研究样本量太小，无法对HIV相关PBL的长期预后进行评论；然而，抗逆转录病毒疗法（ART）与标准化疗联合应用可能会提高生存率。
