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Difference between revisions of "Myeloproliferative neoplasms"
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Myeloproliferative neoplasms (view source)
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'''Myeloproliferative neoplasms''', also '''myeloproliferative disorders''', are a group of indolent hematologic neoplasms. | '''Myeloproliferative neoplasms''', also '''myeloproliferative disorders''', are a group of indolent hematologic neoplasms characterized by clonal expansion of pluripotent hematopoietic progenitor. | ||
These should '''not''' be confused with ''[[myelodysplastic syndromes]]'' (MDS). | These should '''not''' be confused with ''[[myelodysplastic syndromes]]'' (MDS). | ||
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*Essential thrombocythemia. | *Essential thrombocythemia. | ||
*Idiopathic myelofibrosis. | *Idiopathic myelofibrosis. | ||
==Clinic== | |||
* moderate to severe anemia | |||
* pale skin | |||
* fatigue | |||
* prolonged bleeding | |||
* enlarged spleen | |||
* increased clotting | |||
==Molecular== | ==Molecular== | ||
A specific JAK2 mutation (Val617Phe) in seen in a group of patients.<ref name=pmid15781101>{{cite journal |author=Baxter EJ, Scott LM, Campbell PJ, ''et al.'' |title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders |journal=Lancet |volume=365 |issue=9464 |pages=1054–61 |year=2005 |pmid=15781101 |doi=10.1016/S0140-6736(05)71142-9 |url=}}</ref><ref name=pmid15860661>{{cite journal |author=Steensma DP, Dewald GW, Lasho TL, ''et al.'' |title=The JAK2 V617F activating tyrosine kinase mutation is an infrequent event in both "atypical" myeloproliferative disorders and myelodysplastic syndromes |journal=Blood |volume=106 |issue=4 |pages=1207–9 |year=2005 |month=August |pmid=15860661 |pmc=1895198 |doi=10.1182/blood-2005-03-1183 |url=}}</ref> | * BCR-ABL1 negative (DDx: [[Leukemia]] (BCR-ABL1 positive)) | ||
* A specific JAK2 mutation (Val617Phe) in seen in a group of patients.<ref name=pmid15781101>{{cite journal |author=Baxter EJ, Scott LM, Campbell PJ, ''et al.'' |title=Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders |journal=Lancet |volume=365 |issue=9464 |pages=1054–61 |year=2005 |pmid=15781101 |doi=10.1016/S0140-6736(05)71142-9 |url=}}</ref><ref name=pmid15860661>{{cite journal |author=Steensma DP, Dewald GW, Lasho TL, ''et al.'' |title=The JAK2 V617F activating tyrosine kinase mutation is an infrequent event in both "atypical" myeloproliferative disorders and myelodysplastic syndromes |journal=Blood |volume=106 |issue=4 |pages=1207–9 |year=2005 |month=August |pmid=15860661 |pmc=1895198 |doi=10.1182/blood-2005-03-1183 |url=}}</ref> | |||
==See also== | ==See also== | ||