Polycythemia Vera Terminating in Refractory Ascites

フォーマット:

論文

責任表示:

Toyama, Kohtaro ; Mitsui, Takeki ; Yokohama, Akihiko ; Saitoh, Takayuki ; Uchiumi, Hideki ; Handa, Hiroshi ; Sakuraya, Masataka ; Murakami, Hirokazu ; Nojima, Yoshihisa ; Tsukamoto, Norifumi

言語:

英語

出版情報:

北関東医学会, 2012-05-01

著者名:

Toyama, Kohtaro

Mitsui, Takeki

Yokohama, Akihiko

Saitoh, Takayuki

Uchiumi, Hideki

Handa, Hiroshi

Sakuraya, Masataka

Murakami, Hirokazu

Nojima, Yoshihisa

Tsukamoto, Norifumi

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掲載情報:

The Kitakanto medical journal = 北関東医学

ISSN:

1343-2826      

巻:

62

通号:

2

開始ページ:

159

終了ページ:

162

バージョン:

VoR

概要:

Journal Article<br />A 64-year-old woman,with more than a 20 year history of polycythemia vera(PV),developed portal\nhypertension,myelofibrosis and extramedullary hematopoiesis accompanied by massive ascites. Portal\nhypertension resulted not only from infiltration of the liver sinusoids by hematopoietic cells but also from\nnodular regenerative hyperplasia of the liver. Wright-stained smears of ascites samples consisted of\nmesothelial cells … and macrophages. However,cultures of mononuclear cells from the ascites showed the\npresence of hematopoietic progenitor cells including megakaryocyte colony formation and burst forming\nunits. The JAK2-V617F mutation was positive in granulocytes. Contrary to other reports, radiation\ntherapy was not effective and severe myelosuppression continued for more than one month. We present\nthe unusual clinical course for this case of PV and discuss the pathophysiology of refractory ascites. 続きを見る