P30 Adult-onset Langerhans cell histiocytosis related secondary biliary sclerosis in a case of cirrhosis of liver

<h3></h3> We report a unique case of adult-onset hepatic Langerhans cell histiocytosis in a patient with cirrhosis, initially misdiagnosed as decompensated NASH cirrhosis. The patient, a middle-aged obese male with a history of metabolic syndrome and fatty liver, presented with progressive jaundice, pruritus, and neurological symptoms. Despite initial management for NASH cirrhosis and autoimmune hepatitis, his condition deteriorated, prompting further investigation due to atypical features such as persistent neurological symptoms, elevated platelet counts, and absence of ascites. Serial liver function tests showed progressive cholestasis, and imaging revealed features of chronic liver disease and sclerosing cholangitis. A repeat review of liver biopsy identified clusters of abnormal cells positive for CD1a and S100, confirming the diagnosis of LCH. Further evaluation with PET-CT and MRI brain demonstrated multifocal involvement, including the brain, while bone marrow and skin biopsies were negative for LCH, and BRAF mutation was absent. Given the involvement of the liver and brain, and the patient‘s cirrhosis, management was challenging. The patient received Cytarabine chemotherapy but developed febrile neutropenia and septic shock. After recovering from sepsis, he opted for palliative care due to the severe adverse effects of chemotherapy. Unfortunately, he later succumbed to acute kidney injury, hyperkalemia, and metabolic acidosis. This case underscores the critical importance of recognizing atypical presentations in cirrhotic patients and highlights the indispensable role of expert hepatic histopathology in diagnosing rare conditions like LCH. The delayed but accurate diagnosis of LCH prevented an inappropriate liver transplant and unnecessary escalation of immunosuppressants, emphasizing the need for clinicians to be vigilant for deviations from the expected clinical course of cirrhosis. Furthermore, it illustrates the complexities in managing LCH with concurrent cirrhosis and brain involvement, particularly in the absence of BRAF mutations, which limits therapeutic options. Early and precise diagnosis is crucial for guiding appropriate treatment strategies and improving patient outcomes. This case also reinforces the necessity of a multidisciplinary approach in managing complex hepatic diseases, integrating hepatology, oncology, and pathology expertise to ensure comprehensive patient care. 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