Successful long-term outcome of the first combined heart and kidney transplant in a patient with systemic Al amyloidosis.

摘要: Simultaneous cardiac and renal involvement is associated with a particularly poor prognosis in patients AL amyloidosis (AL-A). We report the first case of successful long-term outcome combined heart kidney transplantation not followed by autologous stem cell patient systemic AL-A. The recipient was 46-year-old man end-stage failure serious context Before transplantation, two courses oral melphalan plus prednisone induced partial hematologic remission, as shown decrease circulating free light chain no improvement or function. underwent rescue treatment. During follow-up period (36 months), plasma dyscrasia remains complete normal lambda levels recurrence amyloid deposition on grafts. This demonstrates that systematically may be considered an additional therapeutic option AL-A severe organ dysfunction remission.