Therapy for immunoglobulin light chain amyloidosis: the new and the old.
An accurate diagnosis of amyloidosis and its subtype classification are essential for disease prognostication and treatment. In primary amyloidosis, overall median survival is approximately 2 years and may be less in patients with cardiomyopathy. Current therapy for primary amyloidosis is suboptimal. Controlled studies suggest that treatment with melphalan and prednisone may provide marginal survival benefit. A more aggressive approach such as autologous hematopoietic stem cell transplantation may offer potential for long-term benefit. Although patients undergoing autologous hematopoietic stem cell transplantation are highly selected, response rates can approach 60%, and patients with amyloidosis who respond to treatment have potential for long-term survival. New treatment modalities that were shown to have antitumor activity in multiple myeloma (high-dose dexamethasone and thalidomide) may also be of therapeutic value in primary amyloidosis. Systemic chemotherapy would not be expected to have any beneficial effect on other forms of amyloid and carries significant risk.
Gertz
Morie A
MA
Division of Hematology and Internal Medicine, Mayo Clinic, 200 First Street, SW, Rochester, MN 55905, USA. gertz.morie@mayo.edu
Lacy
Martha Q
MQ
Dispenzieri
Angela
A
D000686:Amyloidosis / Q000145:classification / Q000188:drug therapy* / Q000628:therapy; D000970:Antineoplastic Agents / Q000627:therapeutic use; D003078:Colchicine / Q000627:therapeutic use; D004121:Dimethyl Sulfoxide / Q000627:therapeutic use; D004232:Diuretics / Q000627:therapeutic use; D006801:Humans; D006973:Hypertension / Q000188:drug therapy; D007147:Immunoglobulin Light Chains / Q000032:analysis*; D016377:Organ Transplantation; D036102:Peripheral Blood Stem Cell Transplantation; D016896:Treatment Outcome
D000970:Antineoplastic Agents; D004232:Diuretics; D007147:Immunoglobulin Light Chains; D003078:Colchicine; D004121:Dimethyl Sulfoxide