진성 적혈구증가증에 의한 간문맥해면종 1예
A Case of Cavernoma of Portal Vein associated with Polycythemia Vera
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적혈구증가증은 만성 골수증식질환으로 서서히 발병하여 만성 경과를 취하며 골수와 간, 비장 같은 골수의 조혈기관에서 적혈구계, 골수구계, 그리고 거핵구계의 과잉 증식으로 인하여 말초혈액에서 적혈구, 백혈구 및 혈소판이 증가하고, 특히 적혈구 용적의 증가가 뚜렷한 혈액질환이다. 이 질환의 특징적인 임상양상과 합병증은 적혈구 용적의 증가와 직접적인 관계가 있으며 따라서 합병증 예방을 위하여 적혈구를 정상범위로 유지해야 한다. 환자가 고령이거나 심혈관질환과 같이 혈전증의 고위험군이 아닌 경우에는 사혈과 같은 간단한 단독 치료만으로도 효과적인 조절이 가능하다. 가장 흔한 합병증은 혈전증으로 30-50%에서 발생하고, 대부분 뇌, 심장, 간, 폐 등에 발생하며, 중요한 사망의 원인이 된다. 혈전이나 다른 원인에 의해 간문맥의 폐쇄가 야기되면 간문맥 주위로 다수의 측부혈관이 발달할 수 있는데 이를 간문맥해면종이라고 한다. 저자들은 복부종괴를 주소로 내원하여 종괴에 대한 검사 중 진성 적혈구증가증에 의한 간문맥해면종으로 진단된 1예를 문헌 고찰과 함께 보고하고자 한다.
Abstract
Cavernoma of the portal vein is defined as a formation of venous channels within or around a previously thrombosed portal vein. We experienced a 50-year-old woman who presented a huge hepatic mass with right upper quadrant dull pain. Abdominal computed tomography showed a huge sponge-like hepatic mass with cavernous transformation of portal vein along the common bile duct and common hepatic duct. She had increased hemoglobin/hematocrit (15.7 g/dL/49.1%) and red blood cell mass (35 mL/kg). Platelet count was 450,000/µL and white blood cell count was 13,500/µL. Erythropoietin level was low normal range (10.2 mU/ mL). Bone marrow biopsy showed a moderately hypercellular marrow and overall cellularity was about 80- 90%. Megakaryocytes were slightly increased in number with abnormal clusterings Myelopoiesis and erythropoiesis were also slightly increased with moderate to severe fibrosis. She was diagnosed as polycythemia vera with cavernous transformation of portal vein. Repeated thrombosis occurred in the leg and the toe and was treated with angioplasty and thrombolytic therapy with phlebotomy.
참고문헌 (17)
- "Polycythemia:diagnosis,pathophysiology and therapy II", 100, pp.327~334 (1969)
- "Polycythemia vera:new clinicopathologic perspectives", 130, pp.1126~1132 (2006)
- "Serum erythropoietin in the diagnosis of polycythaemia and after phlebotomy treatment", 81, pp.603~606 (1992)
- "Treatment of polycythemia vera", 166 (1995), Saunders
- "Complications and causes of death in polycythaemia vera", 172, pp.513~523 (1962)
- "Decreased stainable marrow iron in chronic granulocytic leukemia", 81, pp.395~399 (1986)
- "identification of cavernous transformation of the portal vein", pp.999~1001
- "Case of enlarged spleen complicated with ascites,both depending upon varicose dilatation and thrombosis of the portal vein", 14, pp.589~599 (1869)
- "Cavernomatous transformation of the portal vein:its relation to Banti’s disease", 6, pp.353~377
- "CT of portal venous occlusion", 159, pp.717~726 (1992)
- "Portal vein thrombosis resulting in portal hypertension in adults", 65, pp.236~243 (1976)
- "Cavernous transformation of the portal vein:", 165, pp.1151~1155 (1995)
- "Vascular endothelial growth factor and thrombopoietin in patients with essential thrombocythemia and polycythemia vera and thrombotic complications", 110, pp.202~203 (2003)
- "A case of polycythemia vera combined with coagulation disorder", 6, pp.247~256 (1989)
- "Cavernous trans-formation of the portal vein:a common manifestation of Behcet’s disease", 90, pp.1476~1479 (1995)
- "Cavernous transformation of the portal vein causing jaundice,presenting in the form of Wilson’s disease", 104, pp.457~458 (2004)
- "Cavernous transformation of the portal vein associated to multiorgan developmental abnormalities", 24, pp.80~83 (2004)