Diagnosis and treatment update of light chain amyloidosis and the existing problems
10.3760/cma.j.issn.1009-9921.2018.02.004
- VernacularTitle:轻链淀粉样变性的诊治更新及存在的问题
- Author:
Zhongxia HUANG
1
;
Wanli HU
Author Information
1. 100043,首都医科大学附属北京朝阳医院西院血液与肿瘤科
- Keywords:
Light chain;
Amyloidosis;
Prognosis;
Anti-plasma cell therapy;
Anti-amyloid therapy
- From:
Journal of Leukemia & Lymphoma
2018;27(2):76-79
- CountryChina
- Language:Chinese
-
Abstract:
Light chain amyloidosis (AL) is a clonal plasma cell disease in which multiple organs of human body are damaged by amyloid misfolded by light chain of immunoglobulin. Its mechanism is still unclear. Clinically, heart failure, renal insufficiency and other organs failure are seen as prominent symptoms for the majority of patients. The main treatments include melphalan combined with dexamethasone or bortezomib or other target new drugs or autologous stem cell transplantation. Efficacy assessment depends on not only the serum free light chain and M protein levels to judge the hematological response, but also pro-brain natriuretic peptide and troponin I as biomarker to assess the heart and other vital organs function for better outcomes. In the era of new drugs, cardiac involvement is a determinant of survival and prognosis of AL patients. The modern AL treatment usually takes new drug targeted therapy against plasma cells, and combines with anti-amyloidosis treatment to clear the accumulation of amyloidosis chaperonin. Despite the promising advances in treatment, many problems need to be resolved.
