Giancarlo C. Stone, MD; Bruce A. Wall, MD; Ina R. Oppliger, MD; Mark H. Wener, MD; Susan L. Jolly, MD; Augusto Aguirre, MD; Regina Dwyer, MD; Peter A. Simkin, MD
An 82-year-old man and a 34-year-old woman developed subacute, obstructive, fatal vasculopathies characterized by extensive crystalline tissue deposits and monoclonal lambda light chain serum components. Cryocrystalglobulinemia was also present in one patient, and the purified crystals contained only lambda light chain dimers. Although the presentation of these patients resembled that of systemic necrotizing vasculitis, histologic evidence of inflammation was lacking and their subsequent rapid clinical deterioration was not altered by corticosteroid therapy, and in one case cyclophosphamide and plasmapheresis. Both patients died within 3 weeks of presentation.
Learn more about subscription options.
Register Now for a free account.
Stone GC, Wall BA, Oppliger IR, Wener MH, Jolly SL, Aguirre A, et al. A Vasculopathy with Deposition of Lambda Light Chain Crystals. Ann Intern Med. 1989;110:275–278. doi: 10.7326/0003-4819-110-4-275
Download citation file:
Published: Ann Intern Med. 1989;110(4):275-278.
Results provided by:
Copyright © 2017 American College of Physicians. All Rights Reserved.
Print ISSN: 0003-4819 | Online ISSN: 1539-3704
Conditions of Use
This PDF is available to Subscribers Only