Postmortal diagnosis of hemophagocytic lymphohistiocytosis in a patient with liver failure of unknown etiology

Case: A 68 year old man was transferred to our transplant center due to liver failure of unknown etiology. Initial elevated liver values have been documented three weeks before. The patient reported on increased fatigability, night sweats and weight loss of 16 kilogram during the past five month. Apart from a Hepatitis A infection during childhood hepatologic medical history was unremarkable. MELD score was 28 on admission. Acute on chronic liver failure was suspected due to results of diagnostic workup. Criteria for high urgent liver transplantation were not met. Diagnostic workup did not reveal any conclusive results.

Course of disease and diagnosis: Despite supportive therapy the patient's condition detoriated rapidly. Different complications arose. Twelve days after admission to our hospital the patient was transferred to the ICU after cardiopulmonary resuscitation, where he died of rapidly progressive disease. Autopsy revealed lymphohistiocytosis and hemophagocytosis in bone marrow and perihepatic lymph nodes. Histopathological examination of the liver showed uncharacteristic moderate portal and periportal hepatitis together with liver cell necrosis, venoocclusive disease and periportal to septal fibrosis. Considering all clinical and diagnostic findings the patient meets five out of eight diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH). So HLH might be considered as cause for liver failure in our patient. An inciting event could not be identified retrospectively.

Conclusion: HLH is a rare cause of liver failure in adults and might be associated with a fulminant course of disease. Therefore diagnosis of HLH should be considered as rare cause of liver failure in patients with liver failure of unknown etiology.