Marak Creticus P, Ponea Ana M, Shim Chang, Shaheen Shagufta, Guddati Achuta K
Division of Pulmonary and Critical Care Medicine, Montefiore Hospital, Albert Einstein College of Medicine, Yeshiva University, New York, N.Y., USA.
Case Rep Oncol. 2013 Jan;6(1):114-8. doi: 10.1159/000346838. Epub 2013 Feb 28.
Primary effusion lymphoma (PEL) is a subset of large B cell lymphomas and has been mostly associated with human immunodeficiency virus infection. Rare cases have been reported in organ transplant recipients and chronic hepatitis C patients. It typically presents as an effusion in the pleural and pericardial spaces but rarely disseminates. However, involvement of the gastrointestinal tract, lymph nodes and bone marrow has been reported. Diagnosis is based on characteristic clinical, histopathological and immunohistochemical features. We present a case with a right atrial mass which tested positive for human herpes virus 8 (HHV-8), CD20, CD30 and lambda light chains and negative for CD138, kappa light chain, PAX5, Epstein-Barr virus, latent membrane protein 1, CD2, CD3, CD8 and CD56. Bilateral pleural effusions and pericardial effusions were noted which tested positive for HHV-8, CD30 and CD45. The patient responded well to the R-EPOCH regimen with complete resolution of the effusions and a significant decrease in the size of the right atrial mass. This case report illustrates the atypical manifestation of PEL as a right atrial mass.
原发性渗出性淋巴瘤（PEL）是大B细胞淋巴瘤的一个亚型，主要与人类免疫缺陷病毒感染相关。在器官移植受者和慢性丙型肝炎患者中也有罕见病例报道。它通常表现为胸腔和心包腔积液，但很少播散。然而，也有胃肠道、淋巴结和骨髓受累的报道。诊断基于特征性的临床、组织病理学和免疫组化特征。我们报告一例右心房肿块病例，该肿块人类疱疹病毒8（HHV-8）、CD20、CD30和λ轻链检测呈阳性，而CD138、κ轻链、PAX5、爱泼斯坦-巴尔病毒、潜伏膜蛋白1、CD2、CD3、CD8和CD56检测呈阴性。双侧胸腔积液和心包积液被发现，HHV-8、CD30和CD45检测呈阳性。该患者对R-EPOCH方案反应良好，积液完全消退，右心房肿块大小显著减小。本病例报告说明了PEL作为右心房肿块的非典型表现。
