Sequential cyclophosphamide-bortezomib-dexamethasone unmasks the harmful cardiac effect of dexamethasone in primary light-chain cardiac amyloidosis.
Chemotherapy combining cyclophosphamide, bortezomib and dexamethasone is widely used in light-chain amyloidosis. The benefit is limited in patients with cardiac amyloidosis mainly because of adverse cardiac events. Retrospective analysis of our cohort showed that 39 patients died with 42% during the first month. A new escalation-sequential regimen was set to improve the outcomes. Nine newly-diagnosed patients were prospectively treated with close monitoring of serum N-terminal pro-brain natriuretic peptide, troponin-T and free light chains. The results show that corticoids may destabilise the heart through fluid retention. Thus, a sequential protocol may be a promising approach to treat these patients.
Lymphoid Malignancies Unit, Henri Mondor Hospital, Créteil, France; Paris XII University, UPEC, Créteil, France; GRC Amyloid Research Institute, IMRB-INSERM U955, and Mondor Amyloidosis Network, Créteil, France.
Fabien
F
Le Bras
Paris XII University, UPEC, Créteil, France; GRC Amyloid Research Institute, IMRB-INSERM U955, and Mondor Amyloidosis Network, Créteil, France; Department of Clinical Immunology, AP-HP, Henri-Mondor Hospital, Créteil, France.
Valerie
V
Molinier-Frenkel
Paris XII University, UPEC, Créteil, France; GRC Amyloid Research Institute, IMRB-INSERM U955, and Mondor Amyloidosis Network, Créteil, France; Department of Cardiology, AP-HP, Henri-Mondor Hospital, Créteil, France; DHU ATVB, Créteil, France.
Aziz
A
Guellich
Lymphoid Malignancies Unit, Henri Mondor Hospital, Créteil, France; GRC Amyloid Research Institute, IMRB-INSERM U955, and Mondor Amyloidosis Network, Créteil, France.
Jehan
J
Dupuis
Lymphoid Malignancies Unit, Henri Mondor Hospital, Créteil, France; GRC Amyloid Research Institute, IMRB-INSERM U955, and Mondor Amyloidosis Network, Créteil, France.
Karim
K
Belhadj
Paris XII University, UPEC, Créteil, France; GRC Amyloid Research Institute, IMRB-INSERM U955, and Mondor Amyloidosis Network, Créteil, France; Department of Cardiology, AP-HP, Henri-Mondor Hospital, Créteil, France; DHU ATVB, Créteil, France.
Soulef
S
Guendouz
Paris XII University, UPEC, Créteil, France; GRC Amyloid Research Institute, IMRB-INSERM U955, and Mondor Amyloidosis Network, Créteil, France; Department of Cardiology, AP-HP, Henri-Mondor Hospital, Créteil, France; DHU ATVB, Créteil, France.
Karima
K
Ayad
Paris Descartes University, Proteomics Platform, Paris, France.
Magali
M
Colombat
Department of Pathology, AP-HP, Henri-Mondor Hospital, Créteil, France.
Nicole
N
Benhaiem
Paris XII University, UPEC, Créteil, France; GRC Amyloid Research Institute, IMRB-INSERM U955, and Mondor Amyloidosis Network, Créteil, France; Department of Cardiology, AP-HP, Henri-Mondor Hospital, Créteil, France; DHU ATVB, Créteil, France.
Claire Marie
CM
Tissot
Laboratory of Pharmacology, AP-HP, Henri-Mondor Hospital, Créteil, France.
Anne
A
Hulin
Department of Hematology, University Hospital of Limoges, Centre de référence de l'amylose AL et des autres maladies par dépôts d'immunoglobuline monoclonale, Limoges, France.
Arnaud
A
Jaccard
Paris XII University, UPEC, Créteil, France; GRC Amyloid Research Institute, IMRB-INSERM U955, and Mondor Amyloidosis Network, Créteil, France; Department of Cardiology, AP-HP, Henri-Mondor Hospital, Créteil, France; DHU ATVB, Créteil, France; Inserm Clinical Investigation Center 1430, Créteil, F-94000, France. Electronic address: thibaud.damy@aphp.fr.
Thibaud
T
Damy
D000368:Aged; D000369:Aged, 80 and over; D000686:Amyloidosis / Q000097:blood / Q000188:drug therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects* / Q000627:therapeutic use; D001145:Arrhythmias, Cardiac / Q000139:chemically induced*; D000069286:Bortezomib / Q000008:administration & dosage; D003520:Cyclophosphamide / Q000008:administration & dosage; D016757:Death, Sudden, Cardiac / Q000209:etiology*; D003907:Dexamethasone / Q000008:administration & dosage / Q000009:adverse effects*; D005260:Female; D006331:Heart Diseases / Q000097:blood / Q000188:drug therapy*; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000097:blood*; D000075363:Immunoglobulin Light-chain Amyloidosis; D007145:Immunoglobulin kappa-Chains / Q000097:blood; D007146:Immunoglobulin lambda-Chains / Q000097:blood; D008297:Male; D008875:Middle Aged; D020097:Natriuretic Peptide, Brain / Q000097:blood; D010446:Peptide Fragments / Q000097:blood; D012189:Retrospective Studies; D020107:Troponin T / Q000097:blood; D014883:Water-Electrolyte Imbalance / Q000139:chemically induced*
D007147:Immunoglobulin Light Chains; D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains; D010446:Peptide Fragments; D020107:Troponin T; C109794:pro-brain natriuretic peptide (1-76); D020097:Natriuretic Peptide, Brain; D000069286:Bortezomib; D003907:Dexamethasone; D003520:Cyclophosphamide
Cardiac amyloidosis; Dexamethasone; Free light-chains; Heart failure; Light-chain (AL) amyloidosis; Mortality mortality; NT-proBNP; Treatment