ABSTRACT: 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.
Polski merkuriusz lekarski: organ Polskiego Towarzystwa Lekarskiego 04/2012; 32(190):217-20.