HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS: CASE SERIES IN INFANTS AND CHILDREN

Background and Objective: Hemophagocytic Lympho Histiocytisis (HLH) is a rare disorder. It can be primary or secondary, despite the existence of diagnostic guidelines it often remains unrecognized. Fa-milail Hemophagocytic Lymphohistiocystisis occurs typically in children under 1 year of age, secondary HLH typically occurs after 6 years of age. The objective of the study was to highlight the clinical prob-lem and increase awareness of the disease in children in order to facilitate diagnosis and treatment. Methods: This retrospective study was conducted over 17 months period from 1.1.2014 to 30.5.2015 in the hematology department of children’s hospital and included all pediatric patients diagnosed with HLH fulfilling 5 out of 8 revised diagnostic criteria guideline of the HLH – 2004 protocol. None of the patients had genetic testing, sCD25 levels or NK cell activity testing. Results: The diagnosis of HLH was made by fulfilling 5 out of 8 clinical and laboratory diagnostic crite-ria for HLH in 12 patients. Their ages ranged from 3 months to 10 years, male:female ratio was 2:1, fever was present in 100% cases, splenomegaly in 100%, cytopenias in two out of three cell lines in 100%, hypertriglyceridemia in 50%, hypofibrinogenemia in 50%, elevated ferritin in 66.6%, hemopha-gocytosis in bone marrow in 83%, HLH was diagnosed in one patient with Chedik – Higashi syndrome, in one patient with malaria, in one patient with typhoid fever and in one case with systemic juvenile idiopathic arthritis (sJIA). Conclusion: HLH presents with a varied clinical manifestations, often under – recognized, thereby contributing to its high morbidity and mortality. Early diagnosis of HLH and underlying condition and appropriate therapy is crucial to start lifesaving management, this being possible only by keeping a high index of suspicion and using diagnostic guidelines.