Definitions:
LCH was confirmed in all cases with histology by the demonstration of CD1a and / or CD207 immunostaining along with supportive evidence of histiocytic infiltration.10 Site of LCH involvement were described clinically as skin rash, seborrhoea like lesions of the scalp, chronic otitis for middle ear, nails infiltrates etc. All patients underwent Fluorodeoxyglucose-positron emission tomography(FDG-PET) scans and involvement of various organs (bone, spleen, lung, brain, lymph nodes) were noted by significant uptake of FDG.
Hepatic involvement is defined as per the European Consortium for Histiocytosis as palpable liver 3 cm below the coastal margin confirmed by ultrasound and liver dysfunction is defined by hyperbilirubinemia (at least 3 times the upper limit of normal) hypoalbuminemia (<30g/dl) , alanine transaminase (ALT) and / or aspartate transaminase (AST) ( more than 3 times the upper limit of normal), gamma glutamyl transpeptidase (GGT) > 2times normal, ascites, edema, or intra hepatic nodular mass.10
Sclerosing cholangitis in LCH was defined either by involvement of extrahepatic/intrahepatic biliary tree with strictures, dilatation, pruning detected on imaging (CT scan or MRI) and / or on liver biopsy with or without elevated gamma glutamyl transpeptidase.11,12 Portal hypertension was defined as the presence of varices on esophagoduodenoscopy or splenomegaly. An active uptake in FDG-PET scan was used as a marker of disease activity in the extrahepatic sites, whereas an absent uptake would indicate disease remission (passive or burnt out disease).13