Bile duct adenocarcinoma mimicking veno-occlusive disease after autologous bone marrow transplantation for acute leukaemia

• Published in: Bone marrow transplantation (Basingstoke) Vol. 21; no. 12; pp. 1275 - 1277
• Main Authors: BECHERER, A, SCHULENBURG, A, BANKL, H. C, SCHÖFL, R, KEIL, F, REITER, E, GREINIX, H. T, PIKULA, B, KALHS, P
• Format: Journal Article
• Language: English
• Published: Basingstoke Nature Publishing Group 01.06.1998
• Subjects: Acute myeloid leukemia; Adenocarcinoma; Adenocarcinoma - diagnosis; Alkaline phosphatase; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Antibiotics; Ascites; Autografts; Autopsies; Autopsy; Bile Duct Neoplasms - diagnosis; Bile ducts; Bilirubin; Biological and medical sciences; Biopsy; Body weight; Bone marrow; Bone marrow transplantation; Bone Marrow Transplantation - adverse effects; Bone marrow, stem cells transplantation. Graft versus host reaction; Cancer; Cholestasis; Fatalities; Female; Gallbladder; Gallbladder diseases; Gastroenterology. Liver. Pancreas. Abdomen; Hepatic Veno-Occlusive Disease - diagnosis; Histocompatibility antigen HLA; Humans; Leukemia; Leukemia, Myeloid, Acute - therapy; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Medical sciences; Middle Aged; Mimicry; Remission; Sepsis; Septic shock; Siderosis; Stem cell transplantation; Transfusions. Complications. Transfusion reactions. Cell and gene therapy; Transplantation; Transplantation, Autologous; Transplants & implants; Tumors; Veno-occlusive disease; Human; Postoperative; Biliary tract; Acute; Leukemia; Cardiovascular disease; Malignant hemopathy; Transplantation; Malignant tumor; Biliary tract disease; Thrombosis; Differential diagnostic; Venous disease; Vascular disease; Case study; Autograft; Bone marrow; Digestive diseases; Adult; Complication; Female; Deep vein
• ISSN: 0268-3369; 1476-5365
• 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.