Bile duct adenocarcinoma mimicking veno-occlusive disease after autologous bone marrow transplantation for acute leukaemia.
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.
First Department of Internal Medicine, Bone Marrow Transplantation Unit, Vienna, Austria.
A
A
Becherer
A
A
Schulenburg
H C
HC
Bankl
R
R
Schöfl
F
F
Keil
E
E
Reiter
H T
HT
Greinix
B
B
Pikula
P
P
Kalhs
D000230:Adenocarcinoma / Q000175:diagnosis*; D001650:Bile Duct Neoplasms / Q000175:diagnosis*; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D005260:Female; D006504:Hepatic Veno-Occlusive Disease / Q000175:diagnosis*; D006801:Humans; D015470:Leukemia, Myeloid, Acute / Q000628:therapy*; D008875:Middle Aged; D014182:Transplantation, Autologous