title: Acute myelofibrosis: response to recombinant human interferon alpha-2a.
abstract: Acute myelofibrosis is a rare myeloproliferative syndrome characterized by bone marrow proliferation of atypical megakaryocytes, poor response to conventional leukemic therapy, and a fulminant clinical course. Because alpha interferon exhibits potent antiproliferative effects against megakaryocyte progenitors and human fibroblast cell lines, we treated two patients with acute myelofibrosis or the related syndrome of acute myelodysplasia with myelofibrosis with recombinant human interferon alpha-2a. Patient 1 received a 12-week course of interferon alpha (1-6 x 10(6) IU/d) after failure of two cytarabine-based chemotherapy regimens. Interferon administration resulted in prompt improvement in symptoms, stabilization of leukocyte count, and a reduction in circulating blast forms. Primary treatment with interferon (1-3 x 10(6) IU/d x 4 weeks) in patient 2 produced complete hematologic recovery with restoration of marrow cellularity and reduced marrow fibrosis. Our findings suggest that interferon alpha may have significant activity in the treatment of patients with acute myelofibrosis.
authors.affiliation: Department of Internal Medicine, Veterans Administration Medical Center, Phoenix, Arizona 85723.
authors.forename: A F
authors.initials: AF
authors.lastname: List
authors.forename: T D
authors.initials: TD
authors.lastname: Kummet
authors.forename: D M
authors.initials: DM
authors.lastname: Kerr
mesh_terms: D000208:Acute Disease; D000368:Aged; D001853:Bone Marrow / Q000473:pathology; D006801:Humans; D007370:Interferon Type I / Q000627:therapeutic use*; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D055728:Primary Myelofibrosis / Q000097:blood / Q000473:pathology / Q000628:therapy*; D011994:Recombinant Proteins
chemical_list: D007370:Interferon Type I; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins