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原发性渗出性淋巴瘤:临床病理学视角

英文原题:Primary Effusion Lymphoma: A Clinicopathologic Perspective.

PubMed 2022/01/30(内容时间) Cancers (Basel) Q2 · IF 4.8(JCR 2025)

研究概要

原发性渗出性淋巴瘤(PEL)是一种罕见的侵袭性B细胞淋巴瘤,通常局限于浆膜腔,随后在无明确肿块的情况下形成积液。

中文摘要

原发性渗出性淋巴瘤(PEL)是一种罕见且侵袭性强的B细胞淋巴瘤,通常局限于浆膜腔,并在无明确肿块的情况下形成积液。部分肿瘤也可发生于浆膜腔外,最常见部位包括腹膜腔、胸膜腔和心包腔。PEL与人疱疹病毒8型(HHV-8,又称卡波西肉瘤相关疱疹病毒[KSHV])感染有关;约80%的病例合并Epstein-Barr病毒(EBV)感染,提示转化潜能可能存在差异,但其致癌机制尚不清楚。多数患者存在不同程度的免疫功能受损,例如人类免疫缺陷病毒(HIV)感染或实体器官移植后状态;即使接受强化治疗,预后仍较差。目前尚无明确的PEL治疗指南,临床常采用CHOP类方案(环磷酰胺、多柔比星、长春新碱和泼尼松)。由于疾病罕见,研究重点正转向实验性个体化和靶向治疗。当前临床试验包括在EPOCH方案中联合来那度胺和利妥昔单抗,以及使用tabelecleucel治疗复发/难治性EBV相关疾病。

展开英文摘要原文

Primary effusion lymphoma (PEL) is a rare, aggressive B-cell lymphoma that usually localizes to serous body cavities to subsequently form effusions in the absence of a discrete mass. Although some tumors can develop in extracavitary locations, the areas most often affected include the peritoneum, pleural space, and the pericardium. PEL is associated with the presence of human herpesvirus 8 (HHV8), also called the Kaposi sarcoma-associated herpesvirus (KSHV), with some variability in transformation potential suggested by frequent coinfection with the Epstein-Barr virus (EBV) (~80%), although the nature of the oncogenesis is unclear. Most patients suffering with this disease are to some degree immunocompromised (e.g., Human immunodeficiency virus (HIV) infection or post-solid organ transplantation) and, even with aggressive treatment, prognosis remains poor. There is no definitive guideline for the treatment of PEL, although CHOP-like regimens (cyclophosphamide, doxorubicin, vincristine, and prednisone) are frequently prescribed and, given the rarity of this disease, therapeutic focus is being redirected to personalized and targeted approaches in the experimental realm. Current clinical trials include the combination of lenalidomide and rituximab into the EPOCH regimen and the treatment of individuals with relapsed/refractory EBV-associated disease with tabelecleucel.

论文信息

作者
Gathers DA、Galloway E、Kelemen K、Rosenthal A、Gibson SE、Munoz J
第一作者单位
Department of Internal Medicine, University of New Mexico, Albuquerque, NM 87131, USA.Mexico
通讯作者单位
Division of Hematology and Oncology, Mayo Clinic, Phoenix, AZ 85054, USA.United States
文献类型
综述
期刊
Cancers2022 Jan 30
原文标识
PubMed 35158997 · DOI 10.3390/cancers14030722