Nóra Rebeka Jávorniczky1, Imre Bodó2, Tamás Masszi3, Gábor Mikala3. 1. Semmelweis Egyetem, Általános Orvostudományi Kar Budapest. 2. Emory University School of Medicine Atlanta, USA. 3. Egyesített Szent István és Szent László Kórház Budapest.
Abstract
INTRODUCTION: Light chain amyloidosis is characterized by extracellular deposition of a fibrillar material derived from immunglobulin light chain fragments. AIM: The aim of the authors was to assess survival depending on cardiac involvement, therapy, and presence of myeloma. METHOD: The authors studied a retrospective cohort of 29 patients with light chain amyloidosis (13 kappa, 16 lambda) treated in their institution between 2005 and 2014. RESULTS: Twenty-one patients had primary amyloidosis, while 8 had coexisting multiple myeloma. One, two and three or more organs were involved in 4, 8, and 17 patients, respectively. Cardiac involvement (22 cases) inversely correlated with survival. Fifteen (52%) patients received chemotherapy only, while 14 (48%) underwent autologous stem cell transplantation with a median survival of 87 and 11.4 months, respectively. Two patients had heart transplantation and survived 70 and 30 months. Median overall survival was 75.8 months. CONCLUSIONS: Cardiac transplantation followed by autologous stem cell transplantation is feasible in selected patients with light chain amyloidosis and heart failure.
INTRODUCTION: Light chain amyloidosis is characterized by extracellular deposition of a fibrillar material derived from immunglobulin light chain fragments. AIM: The aim of the authors was to assess survival depending on cardiac involvement, therapy, and presence of myeloma. METHOD: The authors studied a retrospective cohort of 29 patients with light chain amyloidosis (13 kappa, 16 lambda) treated in their institution between 2005 and 2014. RESULTS: Twenty-one patients had primary amyloidosis, while 8 had coexisting multiple myeloma. One, two and three or more organs were involved in 4, 8, and 17 patients, respectively. Cardiac involvement (22 cases) inversely correlated with survival. Fifteen (52%) patients received chemotherapy only, while 14 (48%) underwent autologous stem cell transplantation with a median survival of 87 and 11.4 months, respectively. Two patients had heart transplantation and survived 70 and 30 months. Median overall survival was 75.8 months. CONCLUSIONS: Cardiac transplantation followed by autologous stem cell transplantation is feasible in selected patients with light chain amyloidosis and heart failure.
Authors: Attila Nemes; Dóra Földeák; Péter Domsik; Anita Kalapos; Róbert Sepp; Zita Borbényi; Tamás Forster Journal: Quant Imaging Med Surg Date: 2015-12