IgM AL amyloidosis: delineating disease biology and outcomes with clinical, genomic and bone marrow morphological features

Surbhi Sidana, Daniel P. Larson, Patricia T. Greipp, Rong He, Ellen D. McPhail, Angela Dispenzieri, David L. Murray, Surendra Dasari, Stephen M. Ansell, Eli Muchtar, Wilson I. Gonsalves, Taxiarchis V. Kourelis, Marina Ramirez-Alvarado, Prashant Kapoor, S. Vincent Rajkumar, Martha Q. Lacy, Francis K. Buadi, Nelson Leung, Robert A. Kyle, Shaji K. KumarRebecca L. King, Morie A. Gertz

Research output: Contribution to journalArticlepeer-review

8 Scopus citations

Abstract

This study evaluates newly diagnosed IgM (6%, n = 75/1174) vs. non-IgM light chain amyloidosis patients. IgM amyloid patients had lower light chains (12.5 vs. 22.5 mg/dL; p < 0.001). Heart (56% vs. 73%, p = 0.002) and >1 organ involvement (31% vs. 44%, p = 0.02) was less common in IgM amyloidosis, while soft tissue and peripheral nerve involvement was more common. t(11;14) was less common (27% vs. 50%, p = 0.008) in IgM amyloidosis. Rates of MYD88L265P and CXCR4WHIM mutation in IgM amyloidosis were 58% (29/50) and 17% (8/46). Diagnosis after hematopathology review in IgM amyloidosis was pure plasma cell neoplasm (PPCN) in 23% (16/70), lymphoplasmacytic neoplasm (LPL) in 63% (44/70) patients, and other (14%). LPL vs. PPCN groups had distinct genetic abnormalities: t(11;14): 0% (0/18) vs. 60% (9/15), p < 0.001; MYD88L265P mutation: 84% (27/32) vs. 0% (0/14), p < 0.001; CXCR4 mutation: 29% (8/28) vs. 0% (0/14), p = 0.04. Overall survival was shorter in IgM AL when stratified by Mayo 2012 stage; stage 1/2 (59 vs. 125.9 months, p = 0.003) and stage 3/4 (6.5 vs. 12.9 months, p = 0.075), likely due to lower hematologic response rates (6 months: 39% vs. 59%, p = 0.008). We characterized two subtypes of IgM amyloidosis (LPL/PPCN). This can aid in therapeutic decision-making, with treatment directed at the clonal disease.

Original languageEnglish (US)
Pages (from-to)1373-1382
Number of pages10
JournalLeukemia
Volume34
Issue number5
DOIs
StatePublished - May 1 2020

ASJC Scopus subject areas

  • Hematology
  • Oncology
  • Cancer Research

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