Treatment of systemic immunoglobulin light chain amyloidosis / 中华皮肤科杂志

ABSTRACT

Systemic immunoglobulin light chain amyloidosis is a protein-misfolding disease caused by the highly organized amyloid fibrillar aggregates that lead to irreversible organ dysfunction. This review summarizes systemic therapies for this disease according to the disease risk stratification. Patients with low-risk systemic immunoglobulin light chain amyloidosis are eligible for chemotherapy combined with autologous hematopoietic stem cell transplantation. Patients with high-risk systemic immunoglobulin light chain amyloidosis can be treated with protease inhibitors (such as bortezomib, carfilzomib and ixazomib) , immunomodulatory agents (such as lenalidomide, pomalidomide) and new immunological agents (such as daratumumab and NEOD001) .