Young Male with secondary Acute Hemophagocytic lymphohistiocytosis

Abstract

Hemophagocytic Lymphohistiocytosis (HLH), also known as Hemophagocytic syndrome (HPS), is a rare life threatening hematologic disorder manifested by clinical findings of extreme inflammation and unregulated immune activation. HLH can occur as a familial or sporadic disorder, and it can be triggered by a variety of events that disrupts immune hemostasis. Infection is a common trigger both in those with a genetic predisposition and in sporadic cases.

Often the greatest barrier to a successful outcome is delay in diagnosis , which is difficult because of the rarity of this syndrome. Here we, present a case of a young male who presented with enteric fever and confirmed as Acute Hemophagocytic syndrome and deterioatated rapidly inspite of quick diagnosis and treatment.