A vasculopathy with deposition of lambda light chain crystals
| Autor: | Susan L. Jolly, Mark H. Wener, Peter A. Simkin, Giancarlo C. Stone, I R Oppliger, Bruce A. Wall, Augusto Aguirre, Regina Dwyer |
|---|---|
| Rok vydání: | 1989 |
| Předmět: |
Adult
Male Vasculitis Pathology medicine.medical_specialty Cyclophosphamide medicine.medical_treatment Immunoglobulin lambda-Chains Immunoglobulin light chain Methylprednisolone Skin Diseases Diagnosis Differential Necrotizing Vasculitis Internal Medicine medicine Humans Vascular Diseases Aged Aged 80 and over Vascular disease business.industry General Medicine Plasmapheresis medicine.disease Cryoglobulinemia Monoclonal Female Kidney Diseases business Crystallization medicine.drug |
| Zdroj: | Annals of internal medicine. 110(4) |
| ISSN: | 0003-4819 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|