How we treat systemic light-chain amyloidosis.
Systemic light-chain (AL) amyloidosis is a multisystem disease characterized by organ toxicity and damage due to monoclonal free light chains, which are produced by a neoplastic clone of plasma cells in bone marrow. Current treatment strategies target the clone in order to decrease the production of the pathologic light chains and thereby stop or reverse organ toxicity and damage. AL amyloidosis remains a formidable and often incurable disease despite treatment options that include corticosteroids, cytotoxic chemotherapy, risk-adapted melphalan, autologous hematopoietic stem cell transplantation, proteasome inhibitors, and immunomodulatory drugs. New and effective treatment approaches that can reverse the organ damage are urgently needed. Physicians and clinical staff should be aware of the importance of providing best supportive care to patients with advanced AL-related organ dysfunction, given the patients' often tenuous hemodynamics and fragile functional status. Organ transplantation has a role in selected clinical situations, and the treating hematologist should be aware of this sometimes-useful option.
Taussig Cancer Institute of Cleveland Clinic, Weston, Florida.
Chakra P
CP
Chaulagain
Tufts Medical Center, Boston, Massachusetts.
Raymond L
RL
Comenzo
D000686:Amyloidosis / Q000175:diagnosis / Q000378:metabolism* / Q000628:therapy*; D003131:Combined Modality Therapy; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000378:metabolism*; D000075363:Immunoglobulin Light-chain Amyloidosis; D012008:Recurrence; D016896:Treatment Outcome
D007147:Immunoglobulin Light Chains