Essential thrombocytosis pathophysiology: Difference between revisions
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==Pathophysiology== | ==Pathophysiology== | ||
As defined by WHO, ET is a clonal proliferation of pluripotent stem cells with predominantly megakaryocytic differentiation.<ref name="pmidPMID: 16879015">{{cite journal| author=Sanchez S, Ewton A| title=Essential thrombocythemia: a review of diagnostic and pathologic features. | journal=Arch Pathol Lab Med | year= 2006 | volume= 130 | issue= 8 | pages= 1144-50 | pmid=PMID: 16879015 | doi=10.1043/1543-2165(2006)130[1144:ET]2.0.CO;2 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16879015 }} </ref> Recent studies have shown that other cell lineages can also be affected to some degree, resulting in their respective cell proliferation. | |||
The pathologic basis for this disease is unknown. However, essential thrombocytosis resembles [[polycythemia vera]] in that cells of the [[megakaryocyte|megakaryocytic]] series are more sensitive to [[growth factors]]. Platelets derived from the abnormal megakaryocytes do not function properly, which contributes to the clinical features of bleeding and thrombosis. | The pathologic basis for this disease is unknown. However, essential thrombocytosis resembles [[polycythemia vera]] in that cells of the [[megakaryocyte|megakaryocytic]] series are more sensitive to [[growth factors]]. Platelets derived from the abnormal megakaryocytes do not function properly, which contributes to the clinical features of bleeding and thrombosis. | ||
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Pathophysiology
As defined by WHO, ET is a clonal proliferation of pluripotent stem cells with predominantly megakaryocytic differentiation.[1] Recent studies have shown that other cell lineages can also be affected to some degree, resulting in their respective cell proliferation.
The pathologic basis for this disease is unknown. However, essential thrombocytosis resembles polycythemia vera in that cells of the megakaryocytic series are more sensitive to growth factors. Platelets derived from the abnormal megakaryocytes do not function properly, which contributes to the clinical features of bleeding and thrombosis.
In 2005, a mutation in the JAK2 kinase (V617F) was found by multiple research groups [2][3] [4] to be associated with essential thrombocytosis in around 30% of cases. JAK2 is a member of the Janus kinase family. This mutation may be helpful in making a diagnosis or as a target for future therapy.
References
- ↑ Sanchez S, Ewton A (2006). "Essential thrombocythemia: a review of diagnostic and pathologic features". Arch Pathol Lab Med. 130 (8): 1144–50. doi:10.1043/1543-2165(2006)130[1144:ET]2.0.CO;2. PMID 16879015 PMID: 16879015 Check
|pmid=value (help). - ↑ Kralovics R, Passamonti F, Buser AS, Teo SS; et al. (2005 Apr 28). "A gain-of-function mutation of JAK2 in myeloproliferative disorders". N Engl J Med. 352 (17): 1779–90. Check date values in:
|date=(help) - ↑ Baxter EJ et al. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. Lancet. 2005;365:1054-61. PMID 15781101
- ↑ Levine RL et al. Activating mutation in the tyrosine kinase JAK2 in polycythemia vera, essential thrombocythemia, and myeloid metaplasia with myelofibrosis. Cancer Cell. 2005;7:387-97. PMID 15837627