Becherer A, Schulenburg A, Bankl H C, Schöfl R, Keil F, Reiter E, Greinix H T, Pikula B, Kalhs P
First Department of Internal Medicine, Bone Marrow Transplantation Unit, Vienna, Austria.
Bone Marrow Transplant. 1998 Jun;21(12):1275-7. doi: 10.1038/sj.bmt.1701253.
A 52-year-old female underwent autologous BMT because of acute myeloid leukaemia FAB M4 in second remission. Since the patient had no HLA-identical sibling she received a purged autologous BM transplant. On day +5 she developed signs of a sepsis syndrome with fluid retention and was treated with broad-spectrum antibiotic therapy. However, her body weight remained high, ascites and an increase of total serum bilirubin and alkaline phosphatase developed. The icterus worsened to a total bilirubin level of 25 mg/100 ml. Sonographic and endoscopic imaging showed a dilated gall bladder but disclosed a post-hepatic cause for the icterus. A transjugular liver biopsy on day +71 revealed severe cholestasis and siderosis. The patient remained aplastic with constantly increased bilirubin levels. On day +73 septic shock syndrome occurred and the patient died of multiorgan failure 3 days later. At autopsy, a highly differentiated bile duct adenocarcinoma at the porta hepatis, so-called Klatskin tumour, was found, explaining the fatal course with intractable cholestasis.
一名52岁女性因急性髓系白血病FAB M4处于第二次缓解期而接受了自体骨髓移植。由于该患者没有HLA匹配的同胞，她接受了净化的自体骨髓移植。在+5天时，她出现了伴有液体潴留的脓毒症综合征迹象，并接受了广谱抗生素治疗。然而，她的体重仍然很高，出现了腹水，总血清胆红素和碱性磷酸酶升高。黄疸加重至总胆红素水平达到25mg/100ml。超声和内镜成像显示胆囊扩张，但未发现肝后性黄疸的原因。在+71天时进行的经颈静脉肝活检显示严重胆汁淤积和含铁血黄素沉着。患者一直处于再生障碍状态，胆红素水平持续升高。在+73天时发生了感染性休克综合征，患者3天后死于多器官功能衰竭。尸检发现肝门处有一个高分化胆管腺癌，即所谓的Klatskin瘤，这解释了伴有难治性胆汁淤积的致命病程。
