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Outcome of patients with systemic light chain amyloidosis with concurrent renal and cardiac involvement
Author(s) -
Badar Talha,
Cornelison Amanda Megan,
Shah Nina D.,
Bashir Qaiser,
Parmar Simrit,
Patel Krina,
Hosing Chitra,
Popat Uday,
Weber Donna M.,
Thomas Sheeba K.,
Shah Jatin J.,
Orlowski Robert Z.,
Champlin Richard E.,
Qazilbash Muzaffar H.
Publication year - 2016
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1111/ejh.12736
Subject(s) - medicine , al amyloidosis , transplantation , cardiac amyloidosis , amyloidosis , chemotherapy , gastroenterology , renal transplant , immunoglobulin light chain , urology , cardiology , immunology , antibody
Cardiac involvement in systemic light chain amyloidosis ( AL ) is generally associated with a worse outcome, especially if other organs are also involved. We sought to determine whether concurrent cardiac and renal involvement were associated with a worse outcome than either organ alone. We identified 129 patients with AL , who received high‐dose chemotherapy followed by autologous hematopoietic stem cell transplantation (auto‐ HCT ) at our institution between 1997 and 2014. Ninety‐nine patients had either renal (group 1: n = 62, 62%), cardiac (group 2: n = 20, 20%), or both cardiac and renal (group 3: n = 17, 17%) involvement. The overall hematological response rate ( CR + VGPR + PR ) post‐auto‐ HCT in groups 1, 2, and 3 was 69%, 74% and 82%, respectively ( P = 0.62). Overall, organ response in groups 1, 2, and 3 was 39%, 42%, and 70%, respectively. The median PFS from auto‐ HCT in groups 1, 2, and 3 was not reached ( NR ), 13.3 and 21 months, respectively ( P = 0.02). The median OS in groups 1, 2, and 3 was 120, 46, and 60 months, respectively ( P = 0.1). In conclusion, median PFS and OS in patients with concurrent cardiac and renal AL were comparable to patients with cardiac AL only, but worse than patients with renal AL .