Abstract

Introduction

Haemophagocytic lymphohistiocytic syndrome is ahyper inflammatory syndrome due to highly stimulated but ineffective immuneprocess. Based on the etiology it is of two types Primary (Genetic) and Secondary or Acquired (infections, autoimmune and malignant diseases)

Adult onset Still’s disease is an autoimmune inflammatory disease most often seen in young adults of age between 16 to 35 years but rarely can present above 35 years also. It effects women slightly more than men. Adult onset Still’s disease is a clinical diagnosis based on recognition and exclusion of other diseases. It generally presents with fever, arthralgia, rash and Splenomegaly.  Here we report a 41 yr old female patient with features of secondary HLH in whom Adult onset Still’s disease was diagnosed as the cause for secondary HLH.