Ten-year follow-up after autologous stem cell transplantation of a patient with immunoglobulin light-chain (AL) amyloidosis with deposits in the heart, liver and gastrointestinal tract.
The prognosis in amyloid light chain (AL)-amyloidosis and multiorgan involvement is poor, with a high-treatment-related mortality after high-dose melphalan and autologous stem cell transplantation (HDM/SCT). Some patients, however, might benefit from the therapy. We report a case of cardiac AL-amyloidosis with multiorgan involvement where the progressive cardiomyopathy was halted after successful treatment with HDM/SCT in 2001. The patient is in an excellent cardiac condition with a good quality of life, receiving treatment with angiotensinogen receptor blockers and a flexible diuretics regimen at follow-up after 10 years.
Department of Clinical Sciences, Ersta Hospital and Karolinska Institutet, Danderyd Hospital, Stockholm, Sweden.
Marit
M
Mejhert
Robert
R
Hast
Benngt
B
Sandstedt
Izabella
I
Janczewska
D000686:Amyloidosis / Q000276:immunology / Q000473:pathology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D005767:Gastrointestinal Diseases / Q000276:immunology / Q000601:surgery*; D006331:Heart Diseases / Q000276:immunology / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D008107:Liver Diseases / Q000276:immunology / Q000601:surgery*; D008558:Melphalan / Q000627:therapeutic use; D008875:Middle Aged; D019653:Myeloablative Agonists / Q000627:therapeutic use; D009206:Myocardium / Q000473:pathology; D033581:Stem Cell Transplantation
D007147:Immunoglobulin Light Chains; D019653:Myeloablative Agonists; D008558:Melphalan