Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management

IF 10.1 1区医学 Q1 HEMATOLOGY

American Journal of Hematology Pub Date : 2024-05-20 DOI:10.1002/ajh.27376

Andres Ramirez-Gamero, Humberto Martínez-Cordero, Brady E. Beltrán, Jorge Florindez, Luis Malpica, Jorge J. Castillo

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Abstract

Disease Overview

Plasmablastic lymphoma (PBL) is a rare CD20-negative aggressive lymphoma with a poor prognosis under standard treatment options. Though PBL is associated with human immunodeficiency virus infection and other immunosuppressed states, it can also affect immunocompetent individuals.

Diagnosis

The diagnosis requires a high clinical suspicion and pathological confirmation. EBER expression and MYC gene rearrangements are frequently detected. The differential diagnosis includes EBV+ diffuse large B-cell lymphoma, extracavitary primary effusion lymphoma, ALK+ DLBCL, and HHV8+ large B-cell lymphoma, among others.

Risk Stratification

Age ≥60 years, advanced clinical stage, and high intermediate and high International Prognostic Index scores are associated with worse survival.

Management

Combination chemotherapy regimens, such as EPOCH, are recommended. The addition of bortezomib, lenalidomide, or daratumumab might improve outcomes. Including PBL patients and their participation in prospective clinical trials is warranted.

Abstract Image

查看原文本刊更多论文

浆细胞性淋巴瘤：2024 年诊断、风险分层和管理更新。

疾病概述浆细胞性淋巴瘤（PBL）是一种罕见的CD20阴性侵袭性淋巴瘤，标准治疗方案的预后较差。虽然浆细胞性淋巴瘤与人类免疫缺陷病毒感染和其他免疫抑制状态有关，但它也可影响免疫功能正常的个体：诊断：诊断需要高度的临床怀疑和病理证实。经常检测到 EBER 表达和 MYC 基因重排。鉴别诊断包括EBV+弥漫大B细胞淋巴瘤、腔外原发性渗出淋巴瘤、ALK+ DLBCL和HHV8+大B细胞淋巴瘤等：风险分层：年龄≥60岁、临床分期晚期、国际预后指数中高分值和高分值与生存率降低有关：建议采用联合化疗方案，如 EPOCH。建议采用联合化疗方案，如 EPOCH。加用硼替佐米、来那度胺或达拉曲单抗可能会改善疗效。应将PBL患者纳入并参与前瞻性临床试验。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

American Journal of Hematology 医学-血液学

CiteScore

15.70

自引率

3.90%

发文量

363

审稿时长

3-6 weeks

期刊介绍： The American Journal of Hematology offers extensive coverage of experimental and clinical aspects of blood diseases in humans and animal models. The journal publishes original contributions in both non-malignant and malignant hematological diseases, encompassing clinical and basic studies in areas such as hemostasis, thrombosis, immunology, blood banking, and stem cell biology. Clinical translational reports highlighting innovative therapeutic approaches for the diagnosis and treatment of hematological diseases are actively encouraged.The American Journal of Hematology features regular original laboratory and clinical research articles, brief research reports, critical reviews, images in hematology, as well as letters and correspondence.

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