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+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.