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.
Ermann et al. (Thu,) studied this question.