Bleeding Propensity in Waldenström Macroglobulinemia: Potential Causes and Evaluation

Abstract

Waldenström Macroglobulinaemia (WM) is a rare, incurable, low-grade, B cell lymphoma. Symptomatic disease commonly results from marrow or organ infiltration and hyperviscosity secondary to IgM paraprotein, manifesting as anaemia, bleeding and neurological symptoms among others. The causes of the bleeding phenotype in WM remain obscure but are likely to involve several intersecting mechanisms. Evidence of defects in platelet function is lacking in the literature, but factors impacting platelet function and coagulation pathways such as hyperviscosity, vascular bed disturbances, abnormal haematopoiesis, acquired von Willebrand Factor (VWF) syndrome and cryoglobulinaemia may contribute to bleeding. Understanding the pathophysiological mechanisms behind bleeding are important, as common WM therapies including chemo-immunotherapy and Bruton’s tyrosine kinase inhibitors, carry attendant bleeding risks. Furthermore, due to the relatively indolent nature of this lymphoma, most patients diagnosed with WM are often older and have one or more comorbidities requiring treatment with anticoagulant or antiplatelet drugs. It is thus important to understand the origin of the WM bleeding phenotype to better stratify patients according to their bleeding risk and thus enhance confidence in clinical decisions regarding treatment management. In this review, we detail the evidence for various contributing factors to the bleeding phenotype in WM and focus on current and emerging diagnostic tools that will aid evaluation and management of bleeding in these patients.

Waldenström macroglobulinemia: 2023 update on diagnosis, risk stratification, and management

“…He was observed until April 2015. At the time of progression his IgM was 11 500 mg/dl and he was found to have acquired von Willebrand disease 1 . He was treated with single‐agent rituximab which failed to produce a minor response.…”