Treatment Outcomes and Overall Survival of Patients With B‐Cell Prolymphocytic Leukemia

Introduction: B-cell prolymphocytic leukemia (B-PLL) is a rare, aggressive leukemic B-cell malignancy historically associated with poor outcomes and recently reclassified in the WHO Fifth Edition but retained as a distinct entity in the ICC. Methods: We analyzed 950 patients labeled as B-PLL in the National Cancer Database (2004-2019), identified using ICD-O-3 morphology code 9833/3. Results: Median age was 72 years, and 72% received systemic therapy at diagnosis. Median overall survival (OS) was poor (2.8 years) and did not improve over time. Patients initially managed with active surveillance had longer OS (7.8 years). Outcomes varied by age, comorbidity, and facility type. Conclusion: Patients historically diagnosed with B-PLL experience persistently poor outcomes, highlighting the real-world clinical consequences of diagnostic heterogeneity and evolving disease classifications.

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Publication Details

Journal
eJHaem
Published
2026-04-30
DOI
https://doi.org/10.1002/jha2.70299
Primary Topic
Chronic Lymphocytic Leukemia Research
Type
article
Field-Weighted Citation Impact
0.00

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article

Treatment Outcomes and Overall Survival of Patients With B‐Cell Prolymphocytic Leukemia

Allison Bock, Lindsey A. Fitzgerald, Victoria A. Vardell, Harsh Shah et al.
eJHaem
Chronic Lymphocytic Leukemia Research
article

Treatment Outcomes and Overall Survival of Patients With B‐Cell Prolymphocytic Leukemia

Allison Bock, Lindsey A. Fitzgerald, Victoria A. Vardell, Harsh Shah, Deborah M. Stephens, Boyu Hu, Daniel A. Ermann
article en

Abstract

Introduction: B-cell prolymphocytic leukemia (B-PLL) is a rare, aggressive leukemic B-cell malignancy historically associated with poor outcomes and recently reclassified in the WHO Fifth Edition but retained as a distinct entity in the ICC. Methods: We analyzed 950 patients labeled as B-PLL in the National Cancer Database (2004-2019), identified using ICD-O-3 morphology code 9833/3. Results: Median age was 72 years, and 72% received systemic therapy at diagnosis. Median overall survival (OS) was poor (2.8 years) and did not improve over time. Patients initially managed with active surveillance had longer OS (7.8 years). Outcomes varied by age, comorbidity, and facility type. Conclusion: Patients historically diagnosed with B-PLL experience persistently poor outcomes, highlighting the real-world clinical consequences of diagnostic heterogeneity and evolving disease classifications.

eJHaemVol. 7(3)
University of North Carolina at Chapel Hill (US), Huntsman Cancer Institute (US)
National Institutes of Health, National Cancer Institute
Openalex Percentile: Top 9%
Chronic Lymphocytic Leukemia Research
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