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Primary myelofibrosis

Disease definition

A rare myeloproliferative neoplasm characterized by stem-cell derived clonal over proliferation of mature myeloid lineages, such as erythrocytes, leukocytes, and megakaryocytes, with variable degrees of megakaryocyte atypia, associated with reticulin and/or collagen bone marrow fibrosis, osteosclerosis, ineffective erythropoiesis, angiogenesis, extramedullary hematopoiesis, and abnormal cytokine expression.

ORPHA:824

Classification level: Disorder
  • Synonym(s):
    • Agnogenic myeloid metaplasia
    • Idiopathic myelofibrosis
    • Myelofibrosis with myeloid metaplasia
    • Osteomyelofibrosis
  • Prevalence: 1-9 / 100 000
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: D47.4
  • OMIM: 254450
  • UMLS: C0001815  C0026987
  • MeSH: -
  • GARD: 8618
  • MedDRA: -

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