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

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  1 organ involvement (31% vs. 44%, p  = 0.02) was less common in IgM amyloidosis, while soft tissue and peripheral nerve invol...

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Veröffentlicht in:Leukemia 2020-05, Vol.34 (5), p.1373-1382
Hauptverfasser: Sidana, Surbhi, Larson, Daniel P., Greipp, Patricia T., He, Rong, McPhail, Ellen D., Dispenzieri, Angela, Murray, David L., Dasari, Surendra, Ansell, Stephen M., Muchtar, Eli, Gonsalves, Wilson I., Kourelis, Taxiarchis V., Ramirez-Alvarado, Marina, Kapoor, Prashant, Rajkumar, S. Vincent, Lacy, Martha Q., Buadi, Francis K., Leung, Nelson, Kyle, Robert A., Kumar, Shaji K., King, Rebecca L., Gertz, Morie A.
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Sprache:eng
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Zusammenfassung: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  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 MYD88 L265P and CXCR4 WHIM 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  
ISSN:0887-6924
1476-5551
DOI:10.1038/s41375-019-0667-6