1. Successful heart transplantation following melphalan plus dexamethasone therapy in systemic AL amyloidosis.
- Author
-
Mignot A, Bridoux F, Thierry A, Varnous S, Pujo M, Delcourt A, Gombert JM, Goujon JM, Favreau F, Touchard G, Herpin D, and Jaccard A
- Subjects
- Amyloidosis etiology, Dexamethasone administration & dosage, Drug Therapy, Combination, Graft Rejection prevention & control, Heart Failure etiology, Humans, Immunoglobulin kappa-Chains analysis, Immunoglobulin lambda-Chains analysis, Male, Melphalan administration & dosage, Middle Aged, Nephrotic Syndrome etiology, Peripheral Nervous System Diseases etiology, Secondary Prevention, Amyloidosis surgery, Dexamethasone therapeutic use, Heart Failure surgery, Heart Transplantation, Melphalan therapeutic use, Paraproteinemias complications
- Abstract
Recurrence in the allograft and progression in other organs increase mortality after cardiac transplantation in AL amyloidosis. Survival may be improved after suppression of monoclonal light chain (LC) production following high dose melphalan and autologous stem cell transplantation (HDM/ASCT). However, because of high treatment related mortality, this tandem approach is restricted to few patients without significant extra-cardiac involvement. A diagnosis of systemic AL amyloidosis was established in a 45-year old patient with congestive heart failure related to restrictive cardiomyopathy, nephrotic syndrome, peripheral neuropathy, postural hypotension, macroglossia, and lambda LC monoclonal gammopathy. After melphalan and dexamethasone (M-Dex) therapy, which resulted in 80% reduction of serum free lambda LC, he underwent orthotopic cardiac transplantation. Two years later, he remains in a sustained hematologic remission, with no evidence of allograft or extra-cardiac amyloid accumulation. M-Dex should be considered as an alternative therapy in AL amyloid heart transplant recipients ineligible for HDM/ASCT.
- Published
- 2008
- Full Text
- View/download PDF