Clinical Characteristics and Treatment Outcome of Waldenstrom Macroglobulinemia: Experience from a Tertiary Cancer Centre

Abstract

Background and objective: Waldenström macroglobulinemia (WM), also known as lymphoplasmacytic lymphoma, is a rare B-cell lymphoproliferative malignancy characterized by the presence of serum monoclonal immunoglobulin M (IgM) protein and lymphoplasmacytic infiltration in the bone marrow. Patients typically present in their seventh decade with symptoms related to bone marrow infiltration or the effects of monoclonal IgM in the bloodstream. This study aimed to analyze the clinical characteristics and treatment outcomes of patients diagnosed with WM.

Materials and methods: This retrospective analysis was conducted on 26 cases of WM treated at a tertiary cancer center.

Results: WM constituted 0.68% of our non-Hodgkin's lymphoma cases. The median age at presentation was 67 years, with a male-to-female ratio of 2:1. Four patients had lymphadenopathy, six had splenomegaly, and four exhibited hyperviscosity. All patients displayed IgM paraproteinemia; the M band was IgM-kappa in 19 patients and IgM-lambda in seven. All patients received histopathological confirmation. According to the International Prognostic Scoring System for WM (IPSS-WM), 12 patients were classified as intermediate risk, 12 as high risk, and 2 as low risk. Among 26 patients, 20 received upfront treatment, while four were initially observed. Treatment indications included cytopenia in ten patients, constitutional symptoms in five, hyperviscosity in four, and symptomatic lymphadenopathy in one. Sixteen patients received rituximab-based chemotherapy, and four patients with hyperviscosity underwent plasmapheresis. The three-year progression-free survival and overall survival rates were 69.4% and 78%, respectively.

Conclusion: WM is a low-grade B-cell lymphoproliferative disorder with an indolent course, often requiring treatment only after prolonged periods. Diagnosis can be challenging due to the lack of distinct diagnostic features. Rituximab-containing regimens represent the standard of care. Newer targeted treatment options hold promise for improving outcomes in this incurable disease.