Hemophagocytic Lymphohistiocytosis Secondary to Varicella Zoster with Acute Liver Failure

Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life threatening syndrome of excessive immune activation. This condition can be inherited or secondary to other diseases. The therapeutic success is directly related to the rapid diagnosis with the patient who has high clinical suspicious index such as febrile illness with cytopenia, liver enzyme derangement, raised serum ferritin and low fibrinogen levels. Infection is a common trigger both in those with a genetic predisposition and in sporadic cases. We report a 31 years old man who presented with diagnosis of severe varicella zoster infection with multiorgan involvement. The laboratory data showed thrombocytopenia, an elevated level of the liver transaminases and coagulopathy. Antiviral therapy along with supportive treatment initiated. However the general condition deteriorated and he eventually required mechanical ventilation with intensive care unit support. He had persistent high spiking temperature, acute liver failure and raised serum ferritin. With the history of steroid contained health supplements ingestion prior to current hospitalization leading to immunosuppressed state, HLH was diagnosed and treatment was directed towards suppressing the overt immune response. Dexamethasone is chosen here because it can cross the blood brain barrier and tapered over the eight week induction. He responded well to treatment and discharged home.