title: Improvement of amyloid-related symptoms after autologous stem cell transplantation in a patient with hepatomegaly, macroglossia and purpura.
abstract: AL amyloidosis was diagnosed in a 56-year-old woman with spontaneous purpura, macroglossia and hepatomegaly, a serum IgGk monoclonal gammopathy and a 25% plasma cell bone marrow infiltration. She was started on high-dose treatment consisting of four monthly cycles of VID chemotherapy, then underwent a stem cell collection after priming with cyclophosphamide + G-CSF. Myeloablative therapy was with melphalan and busulfan. Hematologic recovery was fast and uncomplicated. At follow-up 22 months from ASCT, the patient shows a complete remission of the clonal plasma cell disorder, normalization of liver size and alkaline phosphatase level and a significant improvement in the signs of vascular and soft tissue amyloid infiltration.
authors.affiliation: Division of Haematology and Department of Bone Marrow Transplantation, Udine University Hospital, Udine, Italy.
authors.forename: F
authors.initials: F
authors.lastname: Patriarca
authors.forename: A
authors.initials: A
authors.lastname: Geromin
authors.forename: R
authors.initials: R
authors.lastname: Fanin
authors.forename: D
authors.initials: D
authors.lastname: Damiani
authors.forename: A
authors.initials: A
authors.lastname: Sperotto
authors.forename: M
authors.initials: M
authors.lastname: Baccarani
mesh_terms: D000686:Amyloidosis / Q000150:complications* / Q000628:therapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002066:Busulfan / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000008:administration & dosage; D003907:Dexamethasone / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D018380:Hematopoietic Stem Cell Transplantation; D006529:Hepatomegaly; D006801:Humans; D015255:Idarubicin / Q000008:administration & dosage; D008260:Macroglossia; D008558:Melphalan / Q000627:therapeutic use; D008875:Middle Aged; D011693:Purpura; D014182:Transplantation, Autologous; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D016179:Granulocyte Colony-Stimulating Factor; D014750:Vincristine; D003907:Dexamethasone; D003520:Cyclophosphamide; D002066:Busulfan; D008558:Melphalan; D015255:Idarubicin