B-Cell Prolymphocytic Leukemia: Clinical Profile and Treatment Outcomes at a Tertiary Cancer Center in South India

Abstract

Introduction: B-cell prolymphocytic leukemia (B-PLL) is a rare lymphoid malignancy considered to have an aggressive course. At diagnosis, most patients have prominent splenomegaly, minor involvement of lymph nodes, and marked leukocytosis with a predominance of prolymphocytes. It is usually treated on similar lines as chronic lymphocytic leukemia. Objectives: The objective of this study was to study the clinicopathological profile and treatment outcomes of B-PLL patients treated at a tertiary cancer center in South India. Materials and Methods: In this retrospective study, the patients diagnosed with B-PLL from August 2020 to July 2022 were included and reviewed for clinical characteristics, pathological findings, and treatment outcomes. The patient's data were collected from hospital medical records and analyzed. Results: Five patients were diagnosed as B-PLL over a period of 2 years. The median age at diagnosis was 52 years. In this series, all the patients presented with high white blood cell (WBC) count ≥50,000 WBC per microliter and splenomegaly was seen in 80%, including massive splenomegaly in 60% of patients. Three patients were treated with bendamustine–rituximab regimen, one patient received single-agent bendamustine, and rest one patient died of tumor lysis syndrome before chemotherapy. Three patients demonstrated partial remission during interim evaluation and rest two patients died of their disease. Conclusion: B-PLL demonstrates a spectrum of clinical features with variation in the extent of the splenomegaly, leukocyte counts, and aggressiveness. This study shows a plethora of clinical features ranging from slowly progressive to rapidly fatal disease.