Incidence and Clinical Presentation of Hemophagocytic Lymphohistiocytosis in Infants: 10 Years' Experience at a Tertiary Care Center

Aim: To document the incidence and clinical presentation of infants diagnosed as Hemophagocytic Lymphohistiocytosis (HLH) in our center. Methods: A retrospective descriptive type of study was conducted and data ofinfants was computed and analyzed, diagnosed as HLH at The Children's Hospital, Lahore, from 2006-2016. We collected the data regarding the gender, clinical presentation and laboratory values. Statistical analysis was performed with the help of IBM SPSS statistics version 22. Results: A total of 57 infants diagnosed as HLH were included during the study period. Amongst them, 37(69.4%) were male, and 20(35%) were female with male to female ratio of 1.8:1. All patients 57(100%) presented with prolonged fever and 53(93%) patients had splenomegaly. Hematological investigations revealed that 34(60%) patients presented with bicytopenia and 18(32%) patients with pancytopenia. Hemophagocytosis in bone marrow was found in 57(100%) patients, deranged LFTs in 54(95%) patients, hyperferritinemia 47(82.4%), hypertriglyceridemia in 40(70%) and hypofibrinoginemia in 24(42%) patients. Conclusion:HLH is a rare but fatal blood disorder that requires early diagnosis on clinical and laboratory grounds for prompt management. The study would help in the early diagnosis of children who presented with fever, abdominal distension and pancytopenia as HLH has a significant mortality rate in the early course of the disease.