BACKGROUND: Patients with light chain (AL) amyloidosis who present with severe heart failure due to cardiac involvement rarely survive more than 6 months. Survival after cardiac transplantation is markedly reduced due to the progression of amyloidosis. Autologous stem-cell transplantation (ASCT) has become a common therapy for AL amyloidosis, but there is an exceedingly high treatment-related mortality in patients with heart failure. METHODS: We developed a treatment strategy of cardiac transplant followed by ASCT. Twenty-six patients were evaluated, and of 18 eligible patients, nine patients underwent cardiac transplantation. Eight of these patients subsequently received an ASCT. RESULTS: Six of seven evaluable patients achieved a complete hematologic remission, and one achieved a partial remission. At a median follow-up of 56 months from cardiac transplant, five of seven patients are alive without recurrent amyloidosis. Their survival is comparable with 17,389 patients who received heart transplants for nonamyloid heart disease: 64% in nonamyloid vs. 60% in amyloid patients at 7 years (P=0.83). Seven of eight transplanted patients have had no evidence of amyloid in their cardiac allograft. CONCLUSIONS: This demonstrates that cardiac transplantation followed by ASCT is feasible in selected patients with AL amyloidosis and heart failure, and that such a strategy may lead to improved overall survival.
BACKGROUND:Patients with light chain (AL) amyloidosis who present with severe heart failure due to cardiac involvement rarely survive more than 6 months. Survival after cardiac transplantation is markedly reduced due to the progression of amyloidosis. Autologous stem-cell transplantation (ASCT) has become a common therapy for AL amyloidosis, but there is an exceedingly high treatment-related mortality in patients with heart failure. METHODS: We developed a treatment strategy of cardiac transplant followed by ASCT. Twenty-six patients were evaluated, and of 18 eligible patients, nine patients underwent cardiac transplantation. Eight of these patients subsequently received an ASCT. RESULTS: Six of seven evaluable patients achieved a complete hematologic remission, and one achieved a partial remission. At a median follow-up of 56 months from cardiac transplant, five of seven patients are alive without recurrent amyloidosis. Their survival is comparable with 17,389 patients who received heart transplants for nonamyloid heart disease: 64% in nonamyloid vs. 60% in amyloid patients at 7 years (P=0.83). Seven of eight transplanted patients have had no evidence of amyloid in their cardiac allograft. CONCLUSIONS: This demonstrates that cardiac transplantation followed by ASCT is feasible in selected patients with AL amyloidosis and heart failure, and that such a strategy may lead to improved overall survival.
Authors: M A Gertz; M Q Lacy; A Dispenzieri; S M Ansell; M A Elliott; D A Gastineau; D J Inwards; I N M Micallef; L F Porrata; A Tefferi; M R Litzow Journal: Bone Marrow Transplant Date: 2004-12 Impact factor: 5.483
Authors: Julian D Gillmore; Hugh J Goodman; Helen J Lachmann; Mark Offer; Ashutosh D Wechalekar; Jayshree Joshi; Mark B Pepys; Philip N Hawkins Journal: Blood Date: 2005-10-06 Impact factor: 22.113
Authors: D C Seldin; N Andrea; I Berenbaum; J L Berk; L Connors; L M Dember; G Doros; S Fennessey; K Finn; S Girnius; A Lerner; C Libbey; H K Meier-Ewert; R O'Connell; C O'Hara; K Quillen; F L Ruberg; F Sam; A Segal; A Shelton; M Skinner; J M Sloan; J F Wiesman; V Sanchorawala Journal: Amyloid Date: 2011-06 Impact factor: 7.141
Authors: A S Renteria; V Sanchorawala; E D Niehaus; F Sun; M J Semigran; D C Seldin Journal: Bone Marrow Transplant Date: 2015-03-09 Impact factor: 5.483
Authors: Jerry D Estep; Arvind Bhimaraj; A M Cordero-Reyes; Brian Bruckner; Matthias Loebe; Guillermo Torre-Amione Journal: Methodist Debakey Cardiovasc J Date: 2012 Jul-Sep