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Review
. 2024 Aug;99(8):1586-1594.
doi: 10.1002/ajh.27376. Epub 2024 May 20.

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

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Free article
Review

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

Andres Ramirez-Gamero et al. Am J Hematol. 2024 Aug.
Free article

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.

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References

REFERENCES

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