Outcome of patients with systemic light chain amyloidosis with concurrent renal and cardiac involvement

Abstract

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.

The Clinical Characteristics and Prognosis of Chinese Patients with Light-Chain Amyloidosis: A Retrospective Multicenter Analysis

“…Badar et al [ 21 ] found that the overall survival of the patients with both cardiac and renal involvement was comparable to that of patients with cardiac involved alone but was worse than that of patients with renal involved alone. We found that the OS of the both cardiac and renal group was better than that of the cardiac alone group but was worse than that of the renal alone group.…”

Clinical Masks of Amyloidosis With the Heart Involvement: Modern Diagnostic Issues

“…сделало его неактуальным. Наши наблюдения подтверждают данные о том, что ATTR сам по себе является факто-ром более благоприятного прогноза [28], в то время как средняя выживаемость при AL типе составляет 46 месяцев [29].…”

The Clinical Characteristics and Prognosis of Chinese Patients With Primary Light-Chain Amyloidosis: A Retrospective Multicenter Analysis

“…Badar et al (20) found that the overall survival of the patients with concurrent cardiac and renal involvement was comparable to that of patients with cardiac involvement only but was worse than that of patients with renal involvement only. We found that the OS of the concurrent cardiac and renal group was better than that of the cardiac group but was worse than that of the renal group.…”