Description
Title: Usefulness of Bruton’s Tyrosine Kinase Inhibitors in Waldenstrom’s Macroglobulinemia (Light Chain Amyloidosis Caused by Lymphoplasmacytic Lymphoma)
Abstract: Only 6 to 10% of patients with the various forms of immunoglobulin-related amyloidosis (AL) have immunoglobulin M (IgM)-related AL, and the majority of these cases are linked to underlying non-lymphoma, Hodgkin’s including Waldenstrom’s macroglobulinemia (WM). Bruton tyrosine kinase (BTK) inhibitors approved for some indolent B cell non-lymphomas Hodgkin’s include ibrutinib, acalabrutinib, and zanubrutinib (NHL). B-cell survival, proliferation, and interaction with the microenvironment are all regulated by the nonreceptor kinase BTK. We looked backward at (n = 4) patients who had underlying WM and AL amyloidosis caused by IgM to assess the safety and efficacy of BTK inhibitor therapy. Patients with AL amyloidosis in the presence of WM responded favorably to the treatment in terms of both hematologic and organ response. Ibrutinib was stopped in one patient due to atrial fibrillation, and acalabrutinib caused a significant thumb hematoma in another patient who required a dose reduction. A good response to BTK inhibitor therapy in our series may be explained by the fact that all patients evaluated had the MYD88 mutation. In larger prospective studies, BTK inhibitors should be further researched for the treatment of AL amyloidosis in patients with lymphoplasmacytic lymphoma/WM.
Paper Quality: SCOPUS / Web of Science Level Research Paper
Subject: Medicine
Sub Category: Hematology
Writer Experience: 20+ Years
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