系统性轻链淀粉样变性患者的支持性护理。
Supportive Care for Patients with Systemic Light Chain Amyloidosis.
机构信息
Hematology/Blood and Marrow Transplantation, Comprehensive Amyloid Program, University of California, 400 Parnassus Avenue, San Francisco, CA 94143, USA.
Division of Hematology/Oncology, Tufts Medical Center, 800 Washington Street, Boston, MA 02111, USA.
出版信息
Hematol Oncol Clin North Am. 2020 Dec;34(6):1177-1191. doi: 10.1016/j.hoc.2020.08.007.
Light chain amyloidosis is a disease in which clonal plasma cells produce toxic immunoglobulin light chains that form amyloid fibrils with deposition in organs, most commonly the heart and kidneys, but also the nervous system, gastrointestinal tract, and soft tissues. Treatment directed at the clonal cells eliminates light chain production and further deposition and may enable organ improvement and decrease the risk of organ failure. Supportive care manages the symptoms of organ involvement and the side effects of treatment. Supportive care also addresses the psychological and social issues that may arise in patients with light chain amyloidosis.
轻链淀粉样变性是一种疾病，其中克隆性浆细胞产生有毒的免疫球蛋白轻链，这些轻链形成淀粉样纤维，并在器官中沉积，最常见于心脏和肾脏，但也存在于神经系统、胃肠道和软组织中。针对克隆细胞的治疗方法可消除轻链的产生和进一步的沉积，并可能使器官得到改善，降低器官衰竭的风险。支持性治疗可控制器官受累的症状和治疗的副作用。支持性治疗还可解决轻链淀粉样变性患者可能出现的心理和社会问题。
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