A 31-year-old man developed diabetes insipidus with urine volume up to 10 to 20 L every 24 hours in 2003.
Four years later, he complained of fatigue, anorexia, jaundice and pruritus, and a symptomatic occipital mass.
Laboratory tests showed an abnormal liver enzyme (Table ​1), the patient was negative for hepatitis viruses.
As shown in Fig.1, abdominal MRI showed multiple low-density lesions in the liver on the T1-weighted image and obvious expansion of the intrahepatic bile duct on the T2-weighted image.
Magnetic resonance cholangiopancreatography revealed multifocal intrahepatic bile duct strictures and dilatation, but the common hepatic duct was normal, it was highly suggestive of SC.
The neurohypophyseal area MRI showed the thickened hypothalamic nuclei and a low-density signal of 4.9 × 5.6 mm in size in the hypothalamic-pituitary area.
A multisystem, high-risk organ LCH was confirmed after occipital mass was biopsied in the local hospital, the patient was given ursodeoxycholic acid 150 mg 3 times a day.
Then, he began to receive a course of COEP chemotherapy (cyclophosphamide, 1000 mg; vincristine, 2 mg; epirubicin, 90 mg; and prednisone, 90 mg) in 2009.
However, on the 5th day of the 1st COEP chemotherapy, the patient appeared to severe liver function injury with an obvious increase of serum bilirubin (Table 1).
He refused further chemotherapy.
One year later, the patient developed liver decompensation with bleeding esophageal varices, ascites, and splenomegaly, and he was referred to LT (Model for End Stage Liver Disease score 17).
He underwent successful orthotopic LT in November 2011 in our center, and the donor came from voluntary deceased citizen organ donation in China.
Liver histopathology after LT revealed micronodular cirrhosis with SC and positive immunostaining (CD1a and S100), suggestive of LCH involving in the liver (Fig.2).
Postoperatively, the man continued to be immunosuppressed with tacrolimus and mycofenolate mofetil.
The patient is currently well with normal liver function and no evidence of recurrence of LCH for 4 and a half years follow-up.