title: Long-lasting partial remission by Interferon-alpha treatment in a child with essential thrombocythemia.
abstract: Essential thrombocythemia (ET) is a clonal myeloproliferative disorder characterized by sustained thrombocytosis, isolated hyperplasia of megakaryocytic lineage, and association with thrombotic or bleeding episodes. It is extremely rare in childhood and frequently presents without evident clinical signs. We describe a 3-year-old girl with severe headache and dizziness suffering from ET, who was treated with Interferon-alpha-2a (IFN) based on the potent effect of this agent to inhibit myeloid colonies induced by phytohemagglutinin A stimulated leukocyte conditioned medium (PHA-LCM). Bone-marrow-derived mononuclear cells of this patient did not exhibit spontaneous colony formation but responded to recombinant human (rh) erythropoietin (EPO), rh granulocyte-colony stimulating factor (G-CSF), granulocyte-macrophage (GM)-CSF, and stem-cell factor in addition to PHA-LCM. After 65 months of in vivo IFN treatment, the patient experienced a sustained partial remission with platelet counts varying between 400 and 600 x 10(3)/microl.
authors.affiliation: Department of Pediatrics, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary.
authors.forename: István
authors.initials: I
authors.lastname: Szegedi
authors.forename: Ilona
authors.initials: I
authors.lastname: Benko
authors.forename: Gabriella
authors.initials: G
authors.lastname: Mero
authors.forename: Agota
authors.initials: A
authors.lastname: Prinzinger
authors.forename: János
authors.initials: J
authors.lastname: Kappelmayer
authors.forename: Csongor
authors.initials: C
authors.lastname: Kiss
mesh_terms: D001856:Bone Marrow Examination; D002675:Child, Preschool; D003114:Colony-Forming Units Assay; D005260:Female; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use*; D010976:Platelet Count; D012074:Remission Induction; D013922:Thrombocytosis / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome
chemical_list: D016898:Interferon-alpha