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

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 (...

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%) lam...

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 ...