Interesting Case of the Month - (IGHP, ICOM) - June
june
Clinical History-
40-year-old male had history of metabolic syndrome with fatty liver,hypothyroidism, dyslipidemia & obesity. He had asymptomatically altered LFT for 4 years, followed by jaundice for 1 year with history of slow activity and forgetfulness forlast 6 months. Current LFT Total Bilirubin-4.5 mg/ dl, AST/ ALT-96/ 256 U/L, SAP/ GGT-190/ 250 U/L, Albumin-3.6 g/ dl. INR-1.2.
The jaundice was painless progressive with itching all over body for about 3months with passage of cholic stool. UGIE-Small esophageal varices.
Radiology-
CECT Abdomen Triple Phase-Features of Chronic Liver Disease and hypodense non enhancing focal lesions likely focal fatty change
PET Scan-Multifocal FDG PET-CT fused axial imageshows diffuse areas of FDG uptake in bothlobes of liver
Microscopic Images-
Special Stains-
Rhodanine showed copper deposits in peri septal hepatocytes and MT stain showing nodularity
Immunohistochemistry –
IHC for HepPar1, CD1a and S100
A. IgG4 Related Sclerosing Cholangitis-
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B. Langerhans Cell Histiocytosis related Sclerosing Cholangitis
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Langerhans cell histiocytosis (LCH) is a rare hematopoietic disorder of unknown pathogenesis characterizedby abnormal proliferation of CD1a-positive dendritic cells. Liver involvement occurs in 10.1% to 18% of multisystem LCH in the paediatric patients, which can result in severe complications, including sclerosing cholangitis (SC) with jaundice. LCH-related SC progresses to biliary cirrhosis more rapidly than primary SC,[3] and have poor response to chemotherapy.Definitive diagnosis requires confirmatory IHC staining with CD1a and S100.Currently, systemic chemotherapy is the mainstay of treatment for hepatic LCH.
C. Cirrhosis NASH Related
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D. Autoimmune Hepatitis
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