浆母细胞淋巴瘤：2024 年关于诊断、风险分层和治疗的更新。
Plasmablastic lymphoma: 2024 update on diagnosis, risk stratification, and management.
机构信息
Division of Hematologic Malignancies, Dana-Farber Cancer Institute, Boston, Massachusetts, USA.
Instituto Nacional de Cancerologia and Hospital Militar Central, Bogota, Colombia.
出版信息
Am J Hematol. 2024 Aug;99(8):1586-1594. doi: 10.1002/ajh.27376. Epub 2024 May 20.
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.
疾病概述
浆母细胞淋巴瘤（PBL）是一种罕见的 CD20 阴性侵袭性淋巴瘤，在标准治疗方案下预后较差。尽管 PBL 与人类免疫缺陷病毒感染和其他免疫抑制状态有关，但它也可能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。通常可检测到 EBER 表达和 MYC 基因重排。鉴别诊断包括 EBV+弥漫性大 B 细胞淋巴瘤、腔外型原发性渗出性淋巴瘤、ALK+DLBCL 和 HHV8+大 B 细胞淋巴瘤等。
风险分层
年龄≥60 岁、晚期临床分期和高、中高危国际预后指数评分与较差的生存相关。
治疗
建议采用 EPOCH 等联合化疗方案。添加硼替佐米、来那度胺或达雷妥尤单抗可能改善预后。应包括 PBL 患者及其参与前瞻性临床试验。
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