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Unusual Clinical Presentation of BCell Prolymphocytic Leukemia Cases as Splenic Marginal Zone Lymphoma Effectively Treated With Rituximab Monotherapy summary
This episode reviews Sachanas et al.'s retrospective case series of five patients with B-cell prolymphocytic leukemia (B-PLL) who presented with massive splenomegaly and prolymphocytosis but whose bone marrow and immunophenotyping were consistent with splenic marginal zone lymphoma (SMZL). The study notes absence of MYC rearrangements or TP53 deletions (one patient had a 7q31 deletion), supporting reclassification of these cases as SMZL variants with prolymphocytic morphology rather than classical B-PLL. Rituximab monotherapy, aligned with SMZL treatment, achieved complete remission in 40% and prolonged responses in 80%, suggesting a safer, well-tolerated option for elderly or frail patients who may not tolerate aggressive chemotherapy. The episode highlights diagnostic challenges and emphasizes comprehensive workup—bone marrow biopsy, immunophenotyping, and cytogenetics—to distinguish SMZL-like prolymphocytic cases from true B-PLL, guiding prognosis and therapy. It discusses limitations such as small sample size and retrospective design, and explores future directions including molecular profiling via next-generation sequencing, routine cytogenetic screening in ambiguous B-cell neoplasms, maintenance Rituximab, and potential integration with targeted therapies (BTK inhibitors, BCL2 antagonists). The takeaway is a move toward precision medicine and personalized, less toxic treatment strategies, with multidisciplinary collaboration essential for optimal care.
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By Pharmacy & Acute Care UniversityThis episode reviews Sachanas et al.'s retrospective case series of five patients with B-cell prolymphocytic leukemia (B-PLL) who presented with massive splenomegaly and prolymphocytosis but whose bone marrow and immunophenotyping were consistent with splenic marginal zone lymphoma (SMZL). The study notes absence of MYC rearrangements or TP53 deletions (one patient had a 7q31 deletion), supporting reclassification of these cases as SMZL variants with prolymphocytic morphology rather than classical B-PLL. Rituximab monotherapy, aligned with SMZL treatment, achieved complete remission in 40% and prolonged responses in 80%, suggesting a safer, well-tolerated option for elderly or frail patients who may not tolerate aggressive chemotherapy. The episode highlights diagnostic challenges and emphasizes comprehensive workup—bone marrow biopsy, immunophenotyping, and cytogenetics—to distinguish SMZL-like prolymphocytic cases from true B-PLL, guiding prognosis and therapy. It discusses limitations such as small sample size and retrospective design, and explores future directions including molecular profiling via next-generation sequencing, routine cytogenetic screening in ambiguous B-cell neoplasms, maintenance Rituximab, and potential integration with targeted therapies (BTK inhibitors, BCL2 antagonists). The takeaway is a move toward precision medicine and personalized, less toxic treatment strategies, with multidisciplinary collaboration essential for optimal care.