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Successful heart transplantation following melphalan plus dexamethasone therapy in systemic AL amyloidosis
- Source :
- Haematologica, Haematologica, Ferrata Storti Foundation, 2008, 93 (3), pp.e32-5. ⟨10.3324/haematol.12108⟩
- Publication Year :
- 2008
- Publisher :
- HAL CCSD, 2008.
-
Abstract
- International audience; 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.
- Subjects :
- Graft Rejection
Male
Melphalan
Nephrotic Syndrome
medicine.medical_treatment
Paraproteinemias
030204 cardiovascular system & hematology
Gastroenterology
Dexamethasone
0302 clinical medicine
Autologous stem-cell transplantation
MESH: Paraproteinemias
Secondary Prevention
Medicine
030212 general & internal medicine
Heart transplantation
MESH: Middle Aged
Peripheral Nervous System Diseases
MESH: Heart Transplantation
Amyloidosis
Hematology
Middle Aged
3. Good health
surgical procedures, operative
MESH: Dexamethasone
MESH: Immunoglobulin lambda-Chains
[SDV.IMM]Life Sciences [q-bio]/Immunology
Drug Therapy, Combination
MESH: Peripheral Nervous System Diseases
medicine.drug
medicine.medical_specialty
MESH: Immunoglobulin kappa-Chains
MESH: Graft Rejection
Immunoglobulin kappa-Chains
03 medical and health sciences
MESH: Melphalan
Immunoglobulin lambda-Chains
Internal medicine
AL amyloidosis
Humans
MESH: Amyloidosis
Heart Failure
MESH: Humans
business.industry
Restrictive cardiomyopathy
medicine.disease
MESH: Male
Surgery
MESH: Recurrence
Transplantation
MESH: Drug Therapy, Combination
Heart failure
MESH: Heart Failure
Heart Transplantation
MESH: Nephrotic Syndrome
business
Subjects
Details
- Language :
- English
- ISSN :
- 03906078 and 15928721
- Database :
- OpenAIRE
- Journal :
- Haematologica, Haematologica, Ferrata Storti Foundation, 2008, 93 (3), pp.e32-5. ⟨10.3324/haematol.12108⟩
- Accession number :
- edsair.doi.dedup.....a6ea01d186cf61a261630e5796af780f