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Primary myelofibrosis

Disease definition

Myelofibrosis with myeloid metaplasia is a myeloproliferative disease with annual incidence of approximately 1 case per 100,000 individuals and age at diagnosis around 60 (an increased prevalence is noted in Ashkenazi Jews). Clinical manifestations depend on the type of blood cell affected and may include anemia, pallor, splenomegaly, hypermetabolic state, petechiae, ecchymosis, bleeding, lymphadenopathy, hepatomegaly, portal hypertension.

ORPHA:824

  • Synonym(s):
    • Agnogenic myeloid metaplasia
    • Idiopathic myelofibrosis
    • Myelofibrosis with myeloid metaplasia
    • Osteomyelofibrosis
  • Prevalence: 1-9 / 100 000
  • Inheritance: -
  • Age of onset: Adult
  • ICD-10: D47.4
  • OMIM: 254450
  • UMLS: C0001815  C0026987
  • MeSH: -
  • GARD: 8618
  • MedDRA: -

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