title: Successful treatment of essential thrombocythemia evolving into agnogenic myeloid metaplasia with interferon-alpha.
abstract: OBJECTIVE
  Essential thrombocythemia and agnogenic myeloid metaplasia as one form of idiopathic myelofibrosis are myeloproliferative disorders and are quite rare in children. A patient with essential thrombocythemia that transformed to "adult-type" agnogenic myeloid metaplasia is described.
  
  
  METHODS
  A routine examination of an 8-year-old girl with bronchial asthma showed thrombocytosis. Essential thrombocythemia was diagnosed 2 years later. During a 3-year follow-up without treatment, transition of the essential thrombocythemia to agnogenic myeloid metaplasia was noticed. The patient had an excellent response to interferon (IFN)-alpha therapy.
  
  
  CONCLUSIONS
  This case is unique because of the availability of premorbid hematologic data and the natural progression of essential thrombocythemia to agnogenic myeloid metaplasia. Whether IFN-alpha therapy can prevent the progression of idiopathic myelofibrosis to leukemia has not yet been determined.
authors.affiliation: Department of Pediatrics, Fukui Medical University School of Medicine, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Kikawa
authors.forename: Y
authors.initials: Y
authors.lastname: Fukumoto
authors.forename: K
authors.initials: K
authors.lastname: Obata
authors.forename: S
authors.initials: S
authors.lastname: Tamura
authors.forename: M
authors.initials: M
authors.lastname: Takeuchi
authors.forename: A
authors.initials: A
authors.lastname: Tanizawa
authors.forename: M
authors.initials: M
authors.lastname: Mayumi
mesh_terms: D002648:Child; D005260:Female; D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use*; D055728:Primary Myelofibrosis / Q000188:drug therapy* / Q000209:etiology*; D013922:Thrombocytosis / Q000150:complications* / Q000503:physiopathology
chemical_list: D016898:Interferon-alpha