Beta 2-microglobulin amyloidosis (AB2M) in patients undergoing long-term hemodialysis. A new type of amyloid.
Amyloidosis has been increasingly recognized in association with renal failure and chronic hemodialysis. This report describes three patients who had long-term hemodialysis (between 7-18 years), in whom deposits developed of a new type of amyloid of beta 2-microglobulin origin. Beta 2-microglobulin amyloid (AB2M) was found in multiple organs, i.e., bone, subendocardium, gastrointestinal blood vessels, tongue, and carpal tunnel connective tissue. AB2M displayed characteristic amyloid features on conventional light and polarized microscopic examination after congo red staining. However immunostaining with anti-amyloid A protein, kappa, and lambda antisera were negative. The studied material reacted positively with beta 2-microglobulin antisera, identifying AB2M in all three cases. Ultrastructural study revealed an unusual curvi-linear fibrillar configuration. AB2M appears to be a new subtype of systemic amyloidosis secondary to renal failure and long-term hemodialysis.
Fuchs
A
A
Jagirdar
J
J
Schwartz
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IS
D000328:Adult; D000686:Amyloidosis / Q000209:etiology* / Q000378:metabolism / Q000473:pathology; D002349:Carpal Tunnel Syndrome / Q000209:etiology / Q000378:metabolism; D004378:Duodenal Diseases / Q000209:etiology; D005269:Femur / Q000032:analysis / Q000473:pathology; D006471:Gastrointestinal Hemorrhage / Q000209:etiology; D006801:Humans; D006973:Hypertension / Q000150:complications; D008297:Male; D008875:Middle Aged; D006435:Renal Dialysis / Q000009:adverse effects*; D013997:Time Factors; D014059:Tongue / Q000032:analysis / Q000473:pathology; D001613:beta 2-Microglobulin / Q000032:analysis*
D001613:beta 2-Microglobulin