Polycythemia Vera Terminating in Refractory Ascites
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概要
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A 64-year-old woman,with more than a 20 year history of polycythemia vera(PV),developed portalhypertension,myelofibrosis and extramedullary hematopoiesis accompanied by massive ascites. Portalhypertension resulted not only from infiltration of the liver sinusoids by hematopoietic cells but also fromnodular regenerative hyperplasia of the liver. Wright-stained smears of ascites samples consisted ofmesothelial cells and macrophages. However,cultures of mononuclear cells from the ascites showed thepresence of hematopoietic progenitor cells including megakaryocyte colony formation and burst formingunits. The JAK2-V617F mutation was positive in granulocytes. Contrary to other reports, radiationtherapy was not effective and severe myelosuppression continued for more than one month. We presentthe unusual clinical course for this case of PV and discuss the pathophysiology of refractory ascites.
- 2012-05-01
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