Rare Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting as Posttransplant Thrombotic Microangiopathy

Abstract

Middle-aged female underwent transplantation with her mother as a donor after appropriate desensitization. The patient developed graft dysfunction with systemic features of thrombotic microangiopathy. Renal allograft biopsy was suggestive of antibody-mediated rejection. She was treated with plasmapheresis followed by intravenous immunoglobulin. However, due to lack of response, further workup was sent and the patient was found to have ADAMTS 13 levels <1% with no evidence of antibodies. Hence, she was diagnosed to have congenital thrombotic thrombocytopenic purpura. A graft nephrectomy was done, and the patient was managed with frozen plasma infusions until remission. Currently, the patient is back on maintenance hemodialysis and is doing well and planned for a genetic study.

Keywords

• plasmapheresis
• posttransplant
• thrombotic microangiopathy