title: Treatment of IgM-associated AL amyloidosis with the combination of rituximab, bortezomib, and dexamethasone.
abstract: IgM-associated AL amyloidosis is a rare clinical entity with distinctive clinical characteristics. Little is known on the applicability of treatment regimens designed for Waldenström's macroglobulinemia (WM) to IgM-AL amyloidosis. Bortezomib is highly effective in AL amyloidosis and the combination of rituximab, bortezomib, and dexamethasone (RBDex) has been successfully tested in WM. Starting in May 2009 we prospectively treated with RBDex 10 patients with IgM-AL amyloidosis. Hematologic response was achieved in 78% of patients, including 3 refractory to previous rituximab. Severe adverse events (grade ≥ 3) were observed in 3 cases. Treatment of IgM-AL amyloidosis with RBDex warrants further investigation in the setting of international clinical trials.
authors: Department of Biochemistry, Amyloidosis Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy.
authors: Giovanni
authors: G
authors: Palladini
authors: Andrea
authors: A
authors: Foli
authors: Paola
authors: P
authors: Russo
authors: Paolo
authors: P
authors: Milani
authors: Laura
authors: L
authors: Obici
authors: Francesca
authors: F
authors: Lavatelli
authors: Giampaolo
authors: G
authors: Merlini
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000188:drug therapy* / Q000276:immunology; D058846:Antibodies, Monoclonal, Murine-Derived / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D001897:Boronic Acids / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D000069286:Bortezomib; D003907:Dexamethasone / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000276:immunology*; D008206:Lymphatic Diseases / Q000188:drug therapy* / Q000276:immunology; D008297:Male; D008875:Middle Aged; D011719:Pyrazines / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D000069283:Rituximab
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001897:Boronic Acids; D007075:Immunoglobulin M; D011719:Pyrazines; D000069283:Rituximab; D000069286:Bortezomib; D003907:Dexamethasone