28248858 Visualization

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