title: High-dose therapy and autologous transplantation in amyloidosis-AL.
abstract: A 53-yr.-old woman with amyloidosis AL was treated with high-dose chemotherapy and autologous stem cell infusion in an attempt to suppress the amyloid secretion. A diagnosis of MGUS had been made six years earlier. During the last year her disease had progressively shifted to a full-blown picture of amyloidosis AL, with renal failure, proteinuria, renal amyloid deposition and plasma cell sheets in the marrow. After an unsuccessful attempt with standard-dose chemotherapy, she received a high-dose regimen of busulphan (14 mg/Kg) and melphalan (40 mg/m2), followed by the infusion of both autologous bone marrow and peripheral blood stem cells. She had full and prompt engraftment, but eight weeks post-graft developed interstitial pneumonitis: CMV was isolated. The patient died while in the intensive care unit. In the literature, this is the first case of amyloidosis AL treated with high-dose therapy and autologous transplantation.
authors.affiliation: Dipartimento di Ematologia, Ospedale V. Cervello, Palermo, Italy.
authors.forename: I
authors.initials: I
authors.lastname: Majolino
authors.forename: R
authors.initials: R
authors.lastname: Marcenò
authors.forename: G
authors.initials: G
authors.lastname: Pecoraro
authors.forename: R
authors.initials: R
authors.lastname: Scimé
authors.forename: S
authors.initials: S
authors.lastname: Vasta
authors.forename: G
authors.initials: G
authors.lastname: Liberti
authors.forename: A
authors.initials: A
authors.lastname: Rizzo
authors.forename: A
authors.initials: A
authors.lastname: Indovina
authors.forename: F
authors.initials: F
authors.lastname: Caronia
mesh_terms: D000686:Amyloidosis / Q000188:drug therapy / Q000209:etiology / Q000628:therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001804:Blood Transfusion, Autologous; D002066:Busulfan / Q000008:administration & dosage; D003131:Combined Modality Therapy; D003907:Dexamethasone; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D008558:Melphalan / Q000008:administration & dosage; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications / Q000188:drug therapy / Q000628:therapy*; D011658:Pulmonary Fibrosis / Q000150:complications; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D003907:Dexamethasone; D004317:Doxorubicin; D002066:Busulfan; D008558:Melphalan