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IgM AL amyloidosis: delineating disease biology and outcomes with clinical, genomic and bone marrow morphological features. Leukemia Sidana, S. n., Larson, D. P., Greipp, P. T., He, R. n., McPhail, E. D., Dispenzieri, A. n., Murray, D. L., Dasari, S. n., Ansell, S. M., Muchtar, E. n., Gonsalves, W. I., Kourelis, T. V., Ramirez-Alvarado, M. n., Kapoor, P. n., Rajkumar, S. V., Lacy, M. Q., Buadi, F. K., Leung, N. n., Kyle, R. A., Kumar, S. K., King, R. L., Gertz, M. A. 2019

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?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?

View details for DOI 10.1038/s41375-019-0667-6

View details for PubMedID 31780812