Young Male with secondary Acute Hemophagocytic lymphohistiocytosis

neeraj kumar tulara

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.


Keywords


Hemophaocytic Lymphohistiocytosis(HLH), Hemophagocytic syndrome (HPS), Enteric (Typhoid) fever

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