Amyloidosis with IgM monoclonal gammopathies.
We sought to review outcomes of patients who have immunoglobulin light-chain amyloidosis of the IgM type. Fifty patients with a serum IgM monoclonal protein and biopsy-proven amyloid were evaluated. The percentages of patients presenting with cardiac, renal, hepatic, and pulmonary amyloid were 44%, 32%, 14%, and 10%, respectively. Forty-two percent had an M-protein spike in the serum greater than 1.5 g/dL, and 14% had an IgM peak greater than 3 g/dL. Amyloidosis was diagnosed easily by biopsies of the subcutaneous fat, rectum, and bone marrow, which demonstrated deposits in 84%, 72%, and 50%, respectively. The median survival of all patients was 24.6 months. Fifty-three percent of all deaths were due to cardiac amyloidosis. Twelve percent died of respiratory failure, and 7% each died of macroglobulinemia, hepatic failure, and renal failure. Of the 22 patients with amyloid cardiomyopathy, the median survival was 11.1 months and fewer than 10% survived 5 years. Of the 28 patients without amyloid cardiomyopathy at diagnosis, the median survival was 27 months, with approximately 30% surviving 5 years. We conclude that the presence of amyloid cardiomyopathy and an increased creatinine concentration had the greatest impact on survival.
Dysproteinemia Clinic and the Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 55905, USA.
Morie A
MA
Gertz
Robert A
RA
Kyle
D000328:Adult; D000368:Aged; D000686:Amyloidosis / Q000209:etiology* / Q000276:immunology / Q000401:mortality; D009202:Cardiomyopathies / Q000209:etiology; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000276:immunology*; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications* / Q000276:immunology; D016019:Survival Analysis; D008258:Waldenstrom Macroglobulinemia / Q000150:complications* / Q000276:immunology
D007075:Immunoglobulin M