Hemophagocytic Lymphohistiocytosis Masquerading as Cholangitis

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare syndrome of unchecked immune activation leading to multi-organ damage with a high mortality. It can be hereditary or triggered by an inflammatory process. Mild hepatitis is present in the majority of patients with HLH, but obstructive jaundice has not been reported. We present the case of a 60 year old male who presented with severe obstructive jaundice from HLH triggered by an underlying lymphoma. A 60 y/o male with a history of DM, CAD and CKD presented with severe jaundice, high fevers and fatigue. His bilirubin was 22 with 20.1 of direct; ALT 33, AST 87, and Alk phos of 114. WBC was 12.4 with left shift. CT abdomen showed mild prominence of CBD and fatty liver without lymphadenopathy. Suspecting cholangitis, antibiotics were started. An MRCP did not show any evidence of biliary dilation or choledocholithiasis and an ERCP showed esophagitis and gastritis but again, no evidence of biliary obstruction. Further work up found a ferritin of 2165 and LDH of 1434. With no lab evidence of hemolytic anemia, a bone marrow biopsy was done, which was inconclusive. Due to progressive liver failure, a liver biopsy was done which showed hemophagocytic histiocytes with evidence of large B cell lymphoma. He had intrahepatic cholestasis due to HLH caused by the lymphoma. Unfortunately with worsening liver and eventual renal failure, he could not receive appropriate chemotherapy, and succumbed to the disease. HLH was considered to be a rare disease in adults; however recently its being increasingly reported, likely due to awareness, rather than rise in incidence. Diagnosis of HLH is a challenge due to nonspecific features mimicking SIRS. Liver injury with mild elevation of transaminases is seen in 85% patients with HLH and about half have hyperbilirubinemia. Florid liver failure is rare, and Post hepatic form of injury mimicking cholangitis has not been reported so far. Prognosis of adult HLH is very poor, with mortality of 41-75% and Delayed diagnosis is considered the most important factor related to treatment failure. Early diagnosis and timely treatment with the HLH-2004 protocol has been shown to improve survival in adult patients with HLH, thus highlighting the importance of recognizing rare presentations of this disease. In patients with liver failure with intrahepatic cholestasis and unclear etiology, HLH should be strongly suspected as timely diagnosis with a Bone marrow or liver biopsy could be life saving.