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Anticardiolipin Antibody-Related Budd-Chiari Syndrome: Report of a Case

抗牛脂蛋白抗體陽性之Budd-Chiari氏症候群:一病例報告

摘要


一位37歲女性病患,主訴上腹痛、漸進性腹脹、氣促、心悸及雙側下肢水腫達一個多月。腹部超音波檢查可見肝靜脈內腔變小、輕度肝脾腫大及中度量腹水。腹部電腦斷層顯示兩側股靜脈,卵巢靜脈及下腔靜脈呈廣範圍栓塞。腹水為漏出液,含血球計數在正常範圍。實驗室檢查則見低白蛋白血症、輕度膽紅素上升及缺鐵性貧血。屬抗磷脂症候群自體抗體之一的抗牛脂蛋白抗體檢查呈陽性反應。肝組織切片則呈肝竇狀隙擴張,合併鄰近肝細胞萎縮,輕度鬱血及Kupffer’s cell 細胞質內含類似血鐵質之物。以上發現合乎Budd -Chiari 氏症候群之診斷條件。我們立即施以靜脈肝素滴注以防止栓塞之進行,腹水則以利尿劑(Spironolac - tone 與Furosemid )成功地控制。經兩週的治療後病人情況良好而出院,並繼續接受口服抗凝血劑Warfarin 的治療及追蹤。

關鍵字

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並列摘要


We report a case of a 37-year-old female who suffered from upper abdominal pain, progressive abdominal distention, shortness of breath, palpitation and pitting edema of lower legs for more than one month. Abdominal sonography showed small caliber of hepatic veins, mild hepatosplenomegaly and moderate ascites. Computed tomography of abdomen disclosed extensive thrombi in bilateral femoral veins, ovarian veins and inferior vena cava. Ascites was transudate with normal cell count. Laboratory data showed hypoalbuminemia, mild elevation of total bilirubin and iron deficiency anemia. Anti-cardiolipin antibody was positive and antinuclear antibody was negative. The histopathological features, including sinusoidal dilatation with atrophic change of adjacent hepatocytes, slight congestion and hemosiderin-like material within the cytoplasm of Kupffer cells, were compatible with tie criteria of BuddChiari syndrome. Heparin was intravenously administered immediately to prevent further progression of thrombosis. The ascites was successfully controlled with diuretics (spironolactone and furosemide). After a two-week course of treatment, she was discharged in good condition and on warfarln anti-coagulant medication.

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