Haematologica, Vol 93, Issue 3, e32-e35 doi:10.3324/haematol.12108
Copyright © 2008 by Ferrata Storti Foundation
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Online Only Articles

Successful heart transplantation following melphalan plus dexamethasone therapy in systemic AL amyloidosis

Aude Mignot1, Frank Bridoux1, Antoine Thierry1, Shaida Varnous2, Myriam Pujo3, Annick Delcourt4, Jean Marc Gombert5, Jean-Michel Goujon6, Fréderic Favreau7, Guy Touchard1, Daniel Herpin8, Arnaud Jaccard9,

1 Department of Nephrology and Renal Transplantation, CHU de Poitiers; Université de Poitiers, France. Centre de référence des amyloses primitives et des autres maladies de dépôts d’immunoglobuline monoclonale
2 Department of Cardiology, Groupe Hospitalier La Pitié Salpétrière, Paris, France
3 Department of Nephrology, Angoulême, France
4 Department of Pathology, Groupe Hospitalier La Pitié Salpétrière, Paris, France
5 Laboratory of Immunology, CHU de Poitiers; Université de Poitiers, France. Centre de référence des amyloses primitives et des autres maladies de dépôts d’immunoglobuline monoclonale
6 Department of Pathology, CHU de Poitiers; Université de Poitiers, France. Centre de référence des amyloses primitives et des autres maladies de dépôts d’immunoglobuline monoclonale
7 Laboratory of Biochemistry and Toxicology, CHU de Poitiers, Université de Poitiers, France
8 Department of Cardiology, CHU de Poitiers; Université de Poitiers, France
9 Department of Clinical Hematology, CHU de Limoges, Université de Limoges, France. Centre de référence des amyloses primitives et des autres maladies de dépôts d’immunoglobuline monoclonale

Correspondence to: Arnaud Jaccard, MD, PhD, Department of Clinical Hematology, CHU Limoges, 1 avenue Martin Luther King, 87000 Limoges, France. Phone: international 33.5 55 05 66 51. Fax: 33.5 55 05 66 49. E-mail: arnaud.jaccard{at}chu-limoges.fr

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.

Key words: AL amyloidosis, cardiac transplantation, melphalan, dexamethasone.




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 Nephrol Dial TransplantHome page
P. Ronco and P. Aucouturier
Renal involvement in AL amyloidosis: the facts, the promise and the hope
Nephrol. Dial. Transplant., October 1, 2009; 24(10): 2967 - 2969.
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