The Kitakanto Medical Journal
Online ISSN : 1881-1191
Print ISSN : 1343-2826
ISSN-L : 1343-2826
CASE REPORTS
Polycythemia Vera Terminating in Refractory Ascites
Kohtaro Toyama, Takeki Mitsui, Akihiko Yokohama, Takayuki Saitoh, Hideki Uchiumi, Hiroshi Handa, Masataka Sakuraya, Hirokazu Murakami, Yoshihisa Nojima, Norifumi Tsukamoto
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Keywords: polycythemia vera, ascites, extramedullary hematopoiesis, nodular regenerative hyperplasia, JAK2-V617F mutation
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2012 Volume 62 Issue 2 Pages 159-162

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Abstract
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.
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© 2012 The Kitakanto Medical Society
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