Crystalglobulinemia causing cutaneous vasculopathy and acute nephropathy in a kidney transplant patient.

Autor: Wilson C; Department of Dermatology, Indiana University School of Medicine, Indianapolis, Indiana, USA., Phillips CL; Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA., Klenk A; Department of Dermatology, Indiana University School of Medicine, Indianapolis, Indiana, USA., Kuhar M; Department of Dermatology, Indiana University School of Medicine, Indianapolis, Indiana, USA.; Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, Indiana, USA., Yaqub MS; Department of Nephrology, Indiana University School of Medicine, Indianapolis, Indiana, USA.
Jazyk: angličtina
Zdroj: American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons [Am J Transplant] 2021 Jun; Vol. 21 (6), pp. 2285-2289. Date of Electronic Publication: 2021 Feb 27.
DOI: 10.1111/ajt.16536
Abstrakt: We present a rare case of crystalglobulinemia causing cutaneous vasculopathy and acute nephropathy in a 66-year-old female kidney transplant recipient. The patient presented with acute kidney injury (AKI), volume overload, anuria, retiform purpura, and blue-black necrosis of her toes. She received a living kidney transplant 7 months earlier with baseline creatinine of 0.6 mg/dl. Transplant kidney biopsy showed massive pseudo-thrombi filling glomerular capillary lumina. Electron microscopy of thrombi revealed an ultrastructural crystalline pattern of linear and curvilinear bundles with ladder-like periodicity typical of crystalglobulin-induced nephropathy. Similar crystalline pseudo-thrombi were detected ultrastructurally in a skin biopsy specimen, indicating systemic involvement. She required several sessions of hemodialysis. Plasmapheresis was initiated to decrease the number of circulating crystalglobulins. In order to treat the underlying paraproteinemia, the patient was started on bortezomib and dexamethasone. After treatment with five cycles of bortezomib, the patient's free kappa to lambda ratio improved to 2.35 from 5.52. Acute kidney injury (AKI) and the cutaneous vasculopathy gradually improved with treatment. This is an extremely rare occurrence of crystalglobulin in a living kidney transplant recipient.
(© 2021 The American Society of Transplantation and the American Society of Transplant Surgeons.)
Databáze: MEDLINE
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