Introduction
Langerhans cell histiocytosis (LCH) is characterised by florid
proliferation of histiocytes causing tissue destruction. Commonly
described in children of age group 1-5 years, this disease also affects
infants and adults.1,2 The spectrum of hepatic
involvement in LCH include hepatomegaly with elevated transaminases,
acute liver failure, secondary sclerosing cholangitis from burnt out
disease, biliary cirrhosis and end stage liver
disease.3-5 Moreover, liver involvement portends a
poorer survival and is classified as high risk
disease.6 Among a large French cohort of 348 patients
with LCH, liver involvement was seen in 14.5% children and their 3-year
survival was 52% when compared to 97% in those without liver
involvement.7
Later, an update from the French cohort involving 1478 children with
LCH, have reported increased survival of these patients, especially
those with risk organ involvement, in the post 1998 era when compared to
pre 1998.8 This improvement is due introduction of
newer chemotherapeutic regimen in cases where the conventional drugs
were showing poor response.8
Precise role of liver transplantation (LT) in the management algorithm
of LCH remains unclear as the literature is sparse. LCH associated end
stage liver disease is one of the clearer indications for
LT.9 However, the need and outcome of LT for other
hepatic manifestations especially in the presence of active disease
remains ambiguous. We also present our management algorithm of LCH
patients with hepatic manifestations, emphasising the importance of a
modified chemotherapeutic protocol and LT in this subset.