A vasculopathy with deposition of lambda light chain crystals #MMPMID2492415
Stone GC; Wall BA; Oppliger IR; Wener MH; Jolly SL; Aguirre A; Dwyer R; Simkin PA
Ann Intern Med 1989[Feb]; 110 (4): 275-8 PMID2492415show ga
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.
Ann+Intern+Med 1989 ; 110 (4): 275-8
