Managing pediatric haemophagocytic lymphohistiocytosis (HLH) in a resource limited setting-a 3 years experience

Abstract

Hemophagocytic Lymphohistiocytosis (HLH) is a rare and fatal entity in children with fever, organomegaly, cytopenias, liver dysfunction and coagulopathy and does not respond to conventional therapies. It is categorized into two types: Primary (inherited) and Secondary (associated with infection, malignancy, autoimmune diseases, etc.). Prognosis of primary HLH is poor, whereas in secondary HLH outcome depends upon the underlying disease. This study is a retrospective analysis of case records of children admitted with a diagnosis of HLH from January 2016 to December 2019 in the Pediatrics department of a teaching hospital in Odisha, India. It describes the clinical features, laboratory findings, diagnosis, treatment and outcome of children with HLH.Thirteen children were diagnosed as HLH for 36 months in the age range from 1 month 11 days to 14 years. Fever and splenomegaly were present in 100% of cases while hepatomegaly in 69.2% and rash in 15.3% of cases. Anaemia, hyperferritinemia were detected in 100% whereas neutropenia in 76.9% and thrombocytopenia in 38.4%. Bone marrow aspiration was done in 7 patients out of whom 6 revealed haemophagocytosis. Serum fibrinogen was low in 8 cases. Out of 13 cases, 7 patients received corticosteroid, and 3 of them also received Cyclosporine along with Steroids. Two cases left against medical advice, 7 had infectious aetiology, and 3 cases were diagnosed as Systemic onset Juvenile Idiopathic arthritis (SoJIA). Seven patients required ICU care. Ten cases recovered, and one died as HLH is a rare entity and has a high chance of fatality early diagnosis and prompt therapy results in a better outcome.