How do you approach treating a patient with IgM-AL amyloidosis?

Topics: Hematologic Malignancies • Medical Oncology • Lymphoma • Myeloma • Amyloidosis • Hematology

Given the rarity of this diagnosis (5-7% of all AL amyloidosis cases), and the prognostic and clinical differences when compared to non IgM-AL amyloidosis, does your therapeutic approach differ between these groups?

Are treatment strategies for Waldenström macroglobulinemia applicable to IgM-AL amyloidosis?

Does the presence of an underlying lymphoplasmacytic lymphoma (LPL) or MYD88 mutation affect your choice of treatment?

Answer from: Medical Oncologist at Academic Institution

Good question. As pointed out, about 5% of lymphomas have a diagnosis of lymphoplasmacytic lymphoma (aka Waldenstroms) instead of a plasma cell dyscrasia. Small burdens of LPL can be difficult to detect unless specific testing is done (e.g., flow for clonal B cells) and intraclonal differentiation ...

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at John Theurer Cancer Center Hackensack Univ Med Center
What conditioning do you use?

Answer from: Medical Oncologist at Academic Institution

This is indeed very rare. My most recent patient presented with nephrotic range proteinuria. SPEP showed IgM lambda. IgM 5799 mg/dl, monoclonal protein 3,693.6 mg/dl, kappa 6.2 mg/l, lambda 10.9 mg/l, K/L 0.57, IgG <75, IgA 24 mg/dl, B2M 3.0, Alb <1.5, 24 hr UPEP: 9,112 mg/day with 319 mg (3.5...

Answer from: Medical Oncologist at Community Practice

IgM-associated AL amyloidosis is a rare but distinct subtype of AL amyloidosis. The rarity of IgM paraprotein-associated AL amyloidosis makes prospective trials difficult and poses a substantial challenge to studying the phenotypic and genetic profiles of the underlying clonal disorder. The largest ...

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