Thrombotic microangiopathy and smoldering multiple myeloma after kidney transplant in a patient with MCP mutation

Abstract

The most frequent cause of atypical hemolytic uremic syndrome (aHUS) is defective regulation of complement activation because of genetic anomalies. We present the case of 53-year-old man with a kidney transplant and stabilized kidney function (creatinine 2.5 mg/dL; proteinuria 0.4 g/24 h) with mycophenolate/tacrolimus/prednisone who was diagnosed of Thrombotic Microangiopathy (TMA). This diagnosis was associated with creatinine and proteinuria rise (3 mg/dL; 2.4 g/24 h) and a new monoclonal IgA/lambda component. Renal biopsy showed membranoproliferative glomerulonephritis; a pathogenic variant in the Membrane cofactor protein (MCP) gene with a polymorphism ggaac, typically associated to secondary aHUS, was identified. We suspected that immunoglobulin could be acting as a trigger for TMA in a genetically susceptible patient, so “clone-directed” therapy with bortezomib and dexamethasone was initiated.