Twenty-one years survival with systemic AL-amyloidosis

AL-amyloidosis has a poor prognosis, typically with cardiac or renal failure ensuing some months after diagnosis. However, sporadically there have been reports of long-term survivors, either with unusual manifestations of amyloidosis, or after concerted chemotherapy to suppress the overt or occult pathological monoclonal plasma cell population responsible for the elaboration of immunoglobulin light chains. We report the case of a 46-year-old man who has survived 21 years after the histological diagnosis of renal amyloidosis was made, after he had presented with severe nephrotic syndrome. This patient was given intensive chemotherapy but came to end-stage renal failure some 10 years later, was dialysed for 1 year, and then was the successful recipient of a cadaveric renal transplant, which is working excellently some 10 years later, with little evidence of recurrent renal or systemic amyloidosis. There is renewed interest in therapy for systemic amyloidosis, and this case demonstrates that with this approach the prognosis can be more favorable than is commonly assumed.