Management Algorithm:
Patients with LCH and hepatic involvement were divided into 2 categories based on the severity of liver involvement.
Category 1: Patients with clinical, radiological or histological evidence of chronic liver disease or cirrhosis with bilirubin <3 and no signs of decompensation i.e absence of ascites, hepatic encephalopathy (HE), or gastrointestinal bleed.
Category 2: Patients with decompensated liver disease; i.e with ascites, variceal bleed or encephalopathy and bilirubin > 3mg/dl, and /or acute on chronic liver failure ; ACLF (bilirubin > 5 mg/dl with INR >1.5 along with onset of ascites and / or HE within 4 weeks of onset of jaundice).14,15.
Category 1 patients were treated with standard chemotherapy regimen for high-risk LCH. whereas category 2 patients who might not tolerate standard chemotherapy regimen were started on modified chemotherapy regimen (Details provided below). 16,17