HLH Syndrome: A Case Report

Sahithi Kilaru, Sadhna Sharma

Abstract: Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome. It is the consequence of a severe, uncontrolled hyperinflammatory reaction that in most cases is triggered by an infectious agent. Frequently affects infants from birth to 18 months of age, but the disease is also observed in children and adults of all ages. Its incidence is estimated to be 1.2 cases per 10, 00, 000 individuals per year. A 42 year old male with fever since 1 month, generalized weakness since 20 days, high colored urine since 5 days with no risk factors, no significant family history and so significant past history. On Examination: Temperature-101*F, Pulse: tachycardia and BP normal. Pallor and icterus were present, hepatosplenomegaly noted, ascites was present and other systems examination was normal. Postinfectious HLH is likely underdiagnosed in critically ill patients. A priority should be placed on rapid evaluation, with the goal of starting treatment as soon as possible as it has an impact on prognosis.

Keywords: lymphohistiocytosis, aggressive, hyperinflammatory, postinfectious