Hemophagocytic Lymphohistiocytosis – Unusual Presentation as Cholangitis- Cholestatic Jaundice

Hemophagocytic lymphohistiocytosis (HLH) is a rare, under diagnosed, fatal and devastating hyper-inflammatory syndrome that has gained increasing recognition over the past decade. HLH can be familial (F-HLH) or acquired; infections, autoimmune diseases, malignancy or immune suppression being the most common triggers of the acquired form. Patients with HLH present with clinical and laboratory evidence of uncontrolled inflammation. They may present with fever, cytopenias, jaundice, and hepatosplenomegaly. Laboratory features may show hyperferritinemia, transaminitis, and low fibrinogen, albumin and natural killer (NK) cells. Delay in diagnosis and management inevitably leads to a rapidly progressive and fatal course. We present a case in which the patient had daily fever, gradually rising bilirubinemia, cytopenias, transaminitis and was initially managed as sepsis, and later as cholangitis and obstructive jaundice before being diagnosed as acquired HLH secondary to Epstein Barr virus associated hepatitis. He succumbed to his illness despite starting therapy for HLH after initial recovery .Delayed diagnosis, refractory progressive HLH and secondary bacterial sepsis were the factors contributing to fatality.