Synonyms
Osteomyelofibrosis; OMF; Chronic idiopathic myelofibrosis; CIMF; Myelofibrosis with myeloid metaplasia; MMM; Agnogenic myeloid metaplasia
Definition and Characteristics
CIMF is an acquired stem-cell disorder, characterized by a secondary, reactive stromal reaction in the bone marrow microenvironment, which is responsible for impaired hematopoesis. In an early hyperproliferative phase proliferative features (thrombocytosis) may be dominant. Later hepatosplenomegaly, cytopenias and extramedullary hematopoesis are prominent features. A small proportion of patients progress to a leukemic transformation [1,2].
Bone marrow fibrosis may be an end-stage feature of another hematological disease (e.g. post-polycythemic (PPMM) or post-thrombocythemic (PTMM) myelofibrosis), sometimes difficult to differentiate from primary IMF.
Prevalence
Estimates of IMF incidence range from 0.5–1.5 new cases/100,000. Given the long survival, prevalence estimates are about 5–10-fold higher.
Molecular and...
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References
Tefferi A, Vardiman JW (2008) Classification and diagnosis of myeloproliferative neoplasms: the 2008 World Health Organization Criteria and point - of - care diagnostic algorithms. Leukemia 22:14–22
Campbell PJ, Green AR (2006) The myeloproliferative disorders. N Engl J Med 355:2452–2466
James C, Ugo V, Le Couédic JP et al. (2005) A unique donal JAK2 mutation leading to constitutive signalling causes polycythacmia vera. Nature 434:1144–1148
Mesa RA, Barosi G, Cervantes F, Reilly JT, Tefferi A (2006) Myelofibrosis with myeloid metaplasia: disease overview and non-transplant treatment options. Best Pract Res Clin Haematol 19:495–517
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© 2009 Springer-Verlag GmbH Berlin Heidelberg
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Schuler, U.S. (2009). Myelofibrosis. In: Lang, F. (eds) Encyclopedia of Molecular Mechanisms of Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-29676-8_1220
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DOI: https://doi.org/10.1007/978-3-540-29676-8_1220
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