Narkhede Mayur, Arora Shagun, Ujjani Chaitra
Lombardi Comprehensive Cancer Center, Georgetown University Hospital, Washington, DC, USA,
Division of Hematology and Oncology, University of California, San Francisco, CA, USA.
Onco Targets Ther. 2018 Jun 28;11:3747-3754. doi: 10.2147/OTT.S167392. eCollection 2018.
Primary effusion lymphoma (PEL) is a rare and aggressive disease, affecting a unique population of patients who are often elderly or immunocompromised. PEL is associated with human herpesvirus type-8 infection and most commonly presents as malignant effusions of the body cavities. Patients diagnosed with PEL often have a compromised immune system from secondary conditions such as HIV. Chemotherapy has traditionally been the cornerstone of treatment for patients with a good performance status and no significant comorbidities. However, an optimal regimen does not exist. Most patients with PEL experience a relapse after frontline therapy within 6-8 months and subsequently require further treatment. In recent years, our understanding of the molecular drivers and environmental factors affecting the pathogenesis of PEL has expanded. This review will discuss the pathogenesis of PEL and various management approaches available in the frontline and relapsed setting as well as targeted agents that have shown promise in this disease.
原发性渗出性淋巴瘤（PEL）是一种罕见且侵袭性强的疾病，影响着一类特殊的患者群体，这些患者通常为老年人或免疫功能低下者。PEL与8型人类疱疹病毒感染相关，最常见的表现为体腔的恶性积液。被诊断为PEL的患者通常因诸如HIV等继发疾病而免疫系统受损。传统上，化疗一直是身体状况良好且无明显合并症患者的治疗基石。然而，目前尚无最佳治疗方案。大多数PEL患者在一线治疗后6 - 8个月内会复发，随后需要进一步治疗。近年来，我们对影响PEL发病机制的分子驱动因素和环境因素的认识有所扩展。本综述将讨论PEL的发病机制、一线和复发情况下可用的各种管理方法以及在该疾病中显示出前景的靶向药物。
