title: Heart transplantation in systemic (AL) amyloidosis: a retrospective study of eight French patients.
abstract: BACKGROUND
  Immunoglobulinic (AL) amyloidosis is a complication of plasma cell dyscrasia, characterized by widespread deposition of amyloid fibrils derived from monoclonal light chains. Cardiac amyloid is the main prognostic factor, with a median survival of six months. Cardiac transplantation in AL amyloidosis is associated with high mortality, due to disease recurrence in the allograft and systemic progression. Suppression of light chain (LC) production with chemotherapy by melphalan plus dexamethasone (MD) or high dose melphalan followed by autologous stem cell transplantation (HDM/ASCT) improves survival. However, both the indications and results of chemotherapy in patients transplanted for cardiac AL amyloidosis remain unclear.
  
  
  OBJECTIVE
  To assess the outcome of cardiac transplantation and haematological therapy in patients with cardiac AL amyloidosis.
  
  
  METHODS
  Eight French patients, who underwent heart transplantation for cardiac AL amyloidosis between 2001 and 2006 were studied retrospectively.
  
  
  RESULTS
  Before transplantation, six patients received MD (n=5) or HDM/ASCT (n=1). Haematological remission was obtained in three patients treated with MD. In the three remaining patients, postoperative HDM/ASCT (n=2) or allogeneic bone marrow transplantation (n=1) resulted in haematological remission in one patient. In 2 patients not treated before transplantation, post-operative treatment with MD resulted in complete hematological remission in one. After a median follow-up of 26 months from cardiac transplantation, six patients were alive and four had sustained haematological remission, as indicated by normal serum free LC levels.
  
  
  CONCLUSIONS
  Appropriate haematological therapy, including MD, may result in a survival benefit in AL amyloidosis patients with advanced heart failure requiring transplantation.
authors.affiliation: Department of Cardiology, CHU of Poitiers, University of Poitiers, Poitiers, France.
authors.forename: Aude
authors.initials: A
authors.lastname: Mignot
authors.forename: Shaida
authors.initials: S
authors.lastname: Varnous
authors.forename: Michel
authors.initials: M
authors.lastname: Redonnet
authors.forename: Arnaud
authors.initials: A
authors.lastname: Jaccard
authors.forename: Eric
authors.initials: E
authors.lastname: Epailly
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Vermes
authors.forename: Pascale
authors.initials: P
authors.lastname: Boissonnat
authors.forename: Iradj
authors.initials: I
authors.lastname: Gandjbakhch
authors.forename: Daniel
authors.initials: D
authors.lastname: Herpin
authors.forename: Guy
authors.initials: G
authors.lastname: Touchard
authors.forename: Frank
authors.initials: F
authors.lastname: Bridoux
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000150:complications / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D009202:Cardiomyopathies / Q000209:etiology / Q000401:mortality / Q000473:pathology / Q000601:surgery*; D003131:Combined Modality Therapy; D003907:Dexamethasone / Q000627:therapeutic use; D005260:Female; D005602:France; D016027:Heart Transplantation; D019650:Hematopoietic Stem Cell Mobilization; D006801:Humans; D008297:Male; D008558:Melphalan / Q000627:therapeutic use; D008875:Middle Aged; D019653:Myeloablative Agonists / Q000627:therapeutic use; D012008:Recurrence; D012189:Retrospective Studies; D033581:Stem Cell Transplantation; D013997:Time Factors; D014182:Transplantation, Autologous; D016896:Treatment Outcome
chemical_list: D019653:Myeloablative Agonists; D003907:Dexamethasone; D008558:Melphalan