Mahl M A, von Schönfeld J, Uppenkamp M, Breuer N
Abtei lungen für Gastroenterologie, Universitätsklinik Essen.
Dtsch Med Wochenschr. 1996 Oct 4;121(40):1226-8. doi: 10.1055/s-2008-1043131.
16 years ago a now 53-year-old woman was found to have primary biliary cirrhosis. 5 years later, after bleeding from oesophageal varices, she had a portacaval shunt. For several years she had been taking ursodeoxycholic acid (750 mg daily). Because of steadily increasing jaundice over the past few years she presented for possible liver transplantation.
There was a discrepancy between the markedly raised serum bilirubin concentration (7.8 mg/dl) and the only slightly raised or normal activities of alkaline phosphatase (247 U/l) and gamma-GT (21 U/l). Further tests confirmed that the patients had not only PBC but also Coombs-negative haemolytic anaemia (haemoglobin 10.7 g/dl, reticulocyte count 122/1000, indirect bilirubin 6.4 mg/dl, haptoglobin not demonstrated, lactate dehydrogenase 316 U/l). She had splenomegaly despite the portacaval shunt. Blood smear revealed spherocytes, but hereditary spherocytosis was not confirmed.
A six-week interruption of taking ursodeoxycholic acid led, as expected, to a rise in the activities of serum alkaline phosphatase and gamma-GT, while haemolysis parameters were not affected.
Serum bilirubin concentration is a decisive prognostic factor in the course of primary biliary cirrhosis and is therefore of particular relevance for the indication of liver transplantation. The reported case demonstrates the importance of considering other causes of hyperbilirubinaemia.
16年前，一名现年53岁的女性被诊断为原发性胆汁性肝硬化。5年后，因食管静脉曲张出血，她接受了门腔分流术。数年来，她一直在服用熊去氧胆酸（每日750毫克）。由于过去几年黄疸持续加重，她前来咨询是否适合进行肝移植。
血清胆红素浓度显著升高（7.8毫克/分升），而碱性磷酸酶（247 U/升）和γ-谷氨酰转移酶（21 U/升）仅轻度升高或正常，两者之间存在差异。进一步检查证实，该患者不仅患有原发性胆汁性肝硬化，还患有抗人球蛋白试验阴性的溶血性贫血（血红蛋白10.7克/分升，网织红细胞计数122/1000，间接胆红素6.4毫克/分升，未检测到触珠蛋白，乳酸脱氢酶316 U/升）。尽管做了门腔分流术，她仍有脾肿大。血涂片显示有球形红细胞，但遗传性球形红细胞增多症未得到证实。
如预期的那样，停用熊去氧胆酸六周导致血清碱性磷酸酶和γ-谷氨酰转移酶活性升高，而溶血参数未受影响。
血清胆红素浓度是原发性胆汁性肝硬化病程中的一个决定性预后因素，因此对于肝移植指征具有特别重要的意义。本报告病例表明，考虑高胆红素血症的其他原因很重要。
