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