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A Case of Cryoglobulinemic Vasculitis: Linking Thymectomy, Autoimmune Disease, and Lymphomagenesis
Zainab Hameed1, Nicholas Carlson1, Mads Hornum1
1Department of Nephrology, Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark.
Abstract:
This case illustrates the possible interplay between autoimmunity, chronic antigenic stimulation, cryoglobulinemic vasculitis, and lymphoproliferative disorders. The patient's progression from myasthenia gravis and total thymectomy to systemic lupus erythematosus, cryoglobulinemic vasculitis, and marginal zone lymphoma demonstrates a continuum of immune dysregulation. Total thymectomy may impair central tolerance, predisposing to secondary autoimmunity and chronic B-cell activation. A 39-year-old woman presented with acute kidney injury, nephrotic-range proteinuria, and pulmonary edema. Laboratory evaluation revealed hypocomplementemia, positive cryoglobulins, a monoclonal IgM band, and elevated rheumatoid factor, findings consistent with cryoglobulinemic vasculitis and immune complex activity. The kidney biopsy demonstrated immune complex-mediated pseudothrombi. She was managed with high-dose corticosteroids, continuous renal replacement therapy, and rituximab, resulting in recovery of renal and cardiac function. During follow-up, she experienced a relapse of cryoglobulinemic vasculitis requiring further rituximab, after which renal function stabilized. This case highlights the complex relationship between thymectomy, autoimmunity, cryoglobulinemic vasculitis, and lymphomagenesis.
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