Abstract Systemic light chain (AL) amyloidosis is a rare, acquired protein misfolding disorder characterized by extracellular deposition of misfolded immunoglobulin light chain fibrils, resulting in organ damage. Treatment is based on anti‐plasma cell regimens derived from multiple myeloma therapy. To date, no approved regimens exist for relapsed/refractory cases. This retrospective multicenter study, performed at 11 centers across Germany, evaluated the efficacy and tolerability of the treatment with teclistamab in 52 patients with relapsed/refractory AL amyloidosis. Hematologic response (≥very good partial response VGPR) was achieved in 81% of patients at Day 15, which increased to 95% at 3 months. Flow‐minimal residual disease (MRD) was negative in 96% (23/24) of cases. Cardiac and renal responses (≥partial response PR) at 6 months were 65% and 78%, respectively. Cytokine release syndrome occurred in 37% of cases, with two classified as Grade 3 and none as Grade 4. Neutropenia Grade 3 or 4 occurred in 5/52 (10%) of patients. The 1‐year overall survival was 83%, and the median overall survival was not reached after a median follow‐up time of 8.8 months. Factors including difference between involved and non‐involved free light chains (dFLC) ≥ 180 mg/L, N‐terminal pro‐brain natriuretic peptide (NTproBNP) ≥ 8500 pg/mL, glomerular filtration rate (GFR) < 20 mL/min/1.73 m 2 , and dialysis did not significantly impact overall survival. In total, nine patients deceased, six of whom deceased due to bacterial infections. Patients receiving immunoglobulin replacement therapy had a 2.01 lower risk of infections Grade 3 or 4 and a 6.14 lower risk of death. Our findings demonstrate the efficacy of teclistamab in a heavily diseased and pretreated cohort with AL amyloidosis and highlight the necessity of a concomitant immunoglobulin replacement therapy.
Carpinteiro et al. (Mon,) studied this question.