[Prognostic factors in light chain amyloidosis]. / Prognosztikai tényezok könnyulánc-amyloidosisban.

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.