TY - JOUR AU - Eli Muchtar, AU - Angela Dispenzieri, AU - Shaji K. Kumar, AU - David Dingli, AU - Martha Q. Lacy, AU - Francis K. Buadi, AU - Suzanne R. Hayman, AU - Prashant Kapoor, AU - Nelson Leung, AU - Rajshekhar Chakraborty, AU - Stephen Russell, AU - John A. Lust, AU - Yi Lin, AU - Ronald S. Go, AU - Steven Zeldenrust, AU - Robert A. Kyle, AU - S. Vincent Rajkumar, AU - Morie A. Gertz, PY - 2016/08/31 Y2 - 2024/03/29 TI - Immunoparesis status in immunoglobulin light chain amyloidosis at diagnosis affects response and survival by regimen type JF - Haematologica JA - haematol VL - 101 IS - 9 SE - Articles DO - 10.3324/haematol.2016.147041 UR - https://haematologica.org/article/view/7823 SP - 1102-1109 AB - Clinical tools to guide in the appropriate treatment selection in immunoglobulin light chain (AL) amyloidosis are not well developed. We evaluated the response and outcome for various regimens at first-line treatment (n=681) and first progression (n=240) stratified by the immunoparesis status at diagnosis. Immunoparesis was assessed by the average relative difference of the uninvolved immunoglobulins, classifying patients into a negative average relative difference (i.e. significant immunoparesis) or a positive average relative difference (no/modest immunoparesis). Treatment was categorized as autologous stem cell transplant and four non-transplant regimens (melphalan-based; bortezomib-based, immunomodulatory drug-based and dexamethasone alone). Patients with significant immunoparesis who underwent stem cell transplant had a significantly lower rate of very good partial response or better response (58%), progression-free survival (median 30 months) and overall survival (108 months), compared to those without significant immunoparesis (80%, 127 months, median not reached, respectively; P