A case of extrahepatic portal vein obstruction due to non-cirrhotic portal vein thrombosis with the JAK2V617F mutation

  • Ishibashi Hiroyuki
    Department of Gastroenterology, Seirei Yokohama General Hospital Department of Medicine and Clinical Oncology, Chiba University Graduate School of Medicine
  • Adachi Seitaro
    Department of Gastroenterology, Seirei Yokohama General Hospital
  • Katakura Yoshiki
    Department of Gastroenterology, Seirei Yokohama General Hospital
  • Fukita Yosho
    Department of Gastroenterology, Seirei Yokohama General Hospital
  • Itobayashi Ei
    Department of Gastroenterology, Asahi General Hospital
  • Yokosuka Osamu
    Department of Medicine and Clinical Oncology, Chiba University Graduate School of Medicine

Bibliographic Information

Other Title
  • JAK2遺伝子変異の関与が疑われた非硬変性門脈血栓症に伴う肝外門脈閉塞症の1例
  • 症例報告 JAK2遺伝子変異の関与が疑われた非硬変性門脈血栓症に伴う肝外門脈閉塞症の1例
  • ショウレイ ホウコク JAK2 イデンシ ヘンイ ノ カンヨ ガ ウタガワレタ ヒコウヘンセイ モンミャク ケッセンショウ ニ トモナウ カン ガイ モンミャク ヘイソクショウ ノ 1レイ

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Abstract

We describe the case of a 45-year-old man, who was pointed out esophageal varices (LmF2CbRCoLg-) by the medical examination in May 2007. The contrast enhanced computed tomography revealed cavernomatous transformation of an extra-hepatic thrombotic obstruction of the portal vein, and splenomegaly. Since he didn't present any other cause for thrombosis, we took a wait-and-see approach. However, during five years follow up period, the portal vein thrombus, splenomegaly and the esophagogastric varices (LsF3CbRC2LgcF2) became progressive. We performed preventive endoscopic sclerotherapy in November 2012. As the number of blood platelets was maintained within a normal level, in spite of the splenomegaly, we suspected the presence of underlying myeloproliferative disease. The bone marrow biopsy was normal, whereas the V617F Janus kinase2 (JAK2V617F) mutation was pointed out. The JAK2V617F mutation was thought to participate in portal vein thrombosis in this case.

Journal

  • Kanzo

    Kanzo 55 (3), 170-175, 2014

    The Japan Society of Hepatology

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