系统性轻链型淀粉样变性，2023年第2版，美国国立综合癌症网络（NCCN）肿瘤学临床实践指南
Systemic Light Chain Amyloidosis, Version 2.2023, NCCN Clinical Practice Guidelines in Oncology.
作者信息
Kumar Shaji K, Callander Natalie S, Adekola Kehinde, Anderson Larry D, Baljevic Muhamed, Campagnaro Erica, Castillo Jorge J, Costello Caitlin, D'Angelo Christopher, Devarakonda Srinivas, Elsedawy Noura, Garfall Alfred, Godby Kelly, Hillengass Jens, Holmberg Leona, Htut Myo, Huff Carol Ann, Hultcrantz Malin, Kang Yubin, Larson Sarah, Lee Hans C, Liedtke Michaela, Martin Thomas, Omel James, Rosenberg Aaron, Sborov Douglas, Valent Jason, Berardi Ryan, Kumar Rashmi
机构信息
Mayo Clinic Cancer Center.
University of Wisconsin Carbone Cancer Center.
出版信息
J Natl Compr Canc Netw. 2023 Jan;21(1):67-81. doi: 10.6004/jnccn.2023.0001.
Primary systemic light chain amyloidosis (SLCA) is characterized by production of light chains that get converted to amyloid fibrils with an affinity for visceral organs and causing organ dysfunction. The therapy for SLCA is directed to recovering the function of the affected organs by targeting the abnormal plasma cell clone and slowing deposition of amyloid fibrils. The NCCN Guidelines for SLCA provide recommendations for workup, diagnosis, and treatment of primary as well as previously treated SLCA.
原发性系统性轻链淀粉样变性（SLCA）的特征是产生轻链，这些轻链会转化为对内脏器官有亲和力的淀粉样纤维，从而导致器官功能障碍。SLCA的治疗旨在通过靶向异常浆细胞克隆并减缓淀粉样纤维的沉积来恢复受影响器官的功能。NCCN SLCA指南为原发性以及既往接受过治疗的SLCA的检查、诊断和治疗提供了建议。
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