[Immunomodulatory drugs in the treatment of primary systemic light chain amyloidosis].
Primary systemic immunoglobulin light chain amyloidosis (AL.) is an incurable clonal plasma cell disorder in which fragments of Ig light chain are deposited in tissues. High dose melphalan and hematopoietic cell transplantation (SCT) is a preferred technique, but only 20% of patients are eligible. Nontransplant candidates can be offered MelDex (melphalan-dexamethasone). Demonstrate comparable efficacy of treatment protocols including immunomodulatory drugs such as TCD (thalidomide, cyclophosphamide, dexamethasone), LMP (lenalidomide, melphalan, prednisone), or bortezomib in combination with dexamethasone. Results of treatment of patients with AL. based on immunomodulatory drugs are promising but require further multicenter clinical trial comparing the MelDex. The main obstacle to effective treatment of AL. still remains a late diagnosis of the disease.
Grzegorz
G
Charliński
Elzbieta
E
Wiater
Wiesław Wiktor
WW
Jedrzejczak
D000686:Amyloidosis / Q000175:diagnosis / Q000188:drug therapy* / Q000276:immunology; D001897:Boronic Acids / Q000008:administration & dosage; D000069286:Bortezomib; D003520:Cyclophosphamide / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D004338:Drug Combinations; D004359:Drug Therapy, Combination; D006801:Humans; D007147:Immunoglobulin Light Chains / Q000276:immunology; D000075363:Immunoglobulin Light-chain Amyloidosis; D007155:Immunologic Factors / Q000627:therapeutic use*; D000077269:Lenalidomide; D008558:Melphalan / Q000008:administration & dosage; D011241:Prednisone / Q000008:administration & dosage; D011719:Pyrazines / Q000008:administration & dosage; D013792:Thalidomide / Q000008:administration & dosage / Q000031:analogs & derivatives
D001897:Boronic Acids; D004338:Drug Combinations; D007147:Immunoglobulin Light Chains; D007155:Immunologic Factors; D011719:Pyrazines; D013792:Thalidomide; D000069286:Bortezomib; D003907:Dexamethasone; D003520:Cyclophosphamide; D000077269:Lenalidomide; D008558:Melphalan; D011241:Prednisone