4 research outputs found
Polycythemia Vera Terminating in Refractory Ascites
A 64-year-old woman,with more than a 20 year history of polycythemia vera(PV),developed portal
hypertension,myelofibrosis and extramedullary hematopoiesis accompanied by massive ascites. Portal
hypertension resulted not only from infiltration of the liver sinusoids by hematopoietic cells but also from
nodular regenerative hyperplasia of the liver. Wright-stained smears of ascites samples consisted of
mesothelial cells and macrophages. However,cultures of mononuclear cells from the ascites showed the
presence of hematopoietic progenitor cells including megakaryocyte colony formation and burst forming
units. The JAK2-V617F mutation was positive in granulocytes. Contrary to other reports, radiation
therapy was not effective and severe myelosuppression continued for more than one month. We present
the unusual clinical course for this case of PV and discuss the pathophysiology of refractory ascites