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EBV+ polymorphic B-cell lymphoproliferative disorder, NOS: a single-center study of a newly recognized pathologic
Suheil Albert Atallah-Yunes1, Thomas M Habermann1, Matthew J Rees1
1Division of Hematology, Mayo Clinic, Rochester, MN, USA.
Blood Cancer Journal
|October 16, 2025
Summary
EBV-positive polymorphic B-cell lymphoproliferative disorder (poly B-LPD) is a new entity. This study characterizes its clinical behavior, finding rituximab effective and highlighting the need for re-biopsy in non-responders.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- EBV-positive polymorphic B-cell lymphoproliferative disorder (poly B-LPD), NOS, is a newly defined entity in the 2022 International Consensus Classification (ICC).
- Its clinical behavior and optimal treatment are poorly understood.
- This study provides initial characterization within the ICC 2022 framework.
Purpose of the Study:
- To retrospectively analyze the clinical characteristics, treatment outcomes, and survival of patients diagnosed with EBV+ poly B-LPD.
- To evaluate the efficacy of reduction of immunosuppression (RIS) and rituximab.
- To identify potential prognostic factors and guide future therapeutic strategies.
Main Methods:
- Retrospective review of 31 patients diagnosed with EBV+ poly B-LPD at Mayo Clinic (2003-2024), excluding transplant recipients.
- Analysis of clinical data, treatment regimens, response rates, event-free survival (EFS), and overall survival (OS).
- Assessment of factors including age, stage, extranodal involvement, autoimmune conditions, and immunosuppressive therapy at diagnosis.
Main Results:
- Median age was 56 years; 71% had extranodal involvement, 68% stage III/IV disease, and 52% autoimmune conditions.
- Reduction of immunosuppression (RIS) led to complete responses in 4/5 patients who underwent it alone, including CNS involvement.
- Rituximab showed efficacy in patients with or without prior immunosuppression. Transformation to DLBCL or T-cell lymphomas occurred in non-responders.
- Median EFS and OS were 8.5 and 8.7 years, respectively. EFS was not significantly influenced by IPI scores, CNS involvement, or baseline immunosuppression.
Conclusions:
- EBV+ poly B-LPD exhibits diverse clinical presentations, with rituximab demonstrating effectiveness.
- Reduction of immunosuppression is a viable initial strategy for patients on therapy.
- Re-biopsy is crucial for patients not responding to initial treatment due to potential transformation.
- Larger studies are needed to validate findings and refine therapeutic guidelines.
