Department of Pathology and Laboratory Medicine, University of California, Irvine, CA, USA.
Am J Clin Pathol. 2013 Aug;140(2):258-73. doi: 10.1309/AJCPHZ3CHO4HUWET.
To report a patient with primary effusion lymphoma who was negative for human herpesvirus-8 (HHV-8), human immunodeficiency virus, Epstein-Barr virus, hepatitis C virus, and hepatitis B virus, as well as review 54 reported cases of HHV-8-unrelated primary effusion lymphoma (PEL)-like lymphoma in the literature to clarify the nature of this entity.
The patients' characteristics, clinical presentation, pathogenesis, morphologic-immunophenotypic features, clinical management, and prognosis were studied.
HHV-8-negative PEL-like lymphomas often occur in immunocompetent and elderly patients, are sometimes associated with chronic inflammation-related fluid overload, are mostly large B-cell or large B-cell with plasmacytic differentiation type, and are associated with a better prognosis.
In various aspects, HHV-8-unrelated PEL-like lymphoma is a different entity from HHV-8-related PEL. Immunophenotype, morphology, and c-myc/8q24 status should be included for differential diagnosis. A test for c-myc or 8q24 abnormalities should be recommended for subdividing HHV-8-unrelated PEL-like lymphoma, which may have benefits in patient management.
报告 1 例人疱疹病毒 8 型（HHV-8）、人类免疫缺陷病毒、EB 病毒、丙型肝炎病毒和乙型肝炎病毒均阴性的原发性渗出性淋巴瘤（PEL）患者，并复习文献中 54 例 HHV-8 无关的 PEL 样淋巴瘤，以明确该实体的性质。
研究了患者的特征、临床表现、发病机制、形态-免疫表型特征、临床管理和预后。
HHV-8 阴性 PEL 样淋巴瘤常发生于免疫功能正常和老年患者中，有时与慢性炎症相关的液体超负荷有关，大多为大 B 细胞或大 B 细胞伴浆细胞分化型，预后较好。
在各个方面，HHV-8 无关的 PEL 样淋巴瘤与 HHV-8 相关的 PEL 是不同的实体。免疫表型、形态和 c-myc/8q24 状态应包括在鉴别诊断中。建议对 HHV-8 无关的 PEL 样淋巴瘤进行 c-myc 或 8q24 异常的检测，这可能有助于患者管理。
