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.