Profile of hemophagocytic lymphohistiocytosis; efficacy of intravenous immunoglobulin therapy.

OBJECTIVE: To study the profile of children with Hemophagocytic lymphohistiocytosis (HLH) and compare the outcome of treatment with intravenous immunoglobulin therapy and Dexamethasone vs. HLH-2004 protocol.
METHODS: The present retrospective cohort study was conducted in a tertiary care pediatric hospital in Chennai. Children with a diagnosis of HLH admitted to the hospital from June 2008 through June 2011 were included. Medical records of the subjects were reviewed and their clinical and demographic profile studied. Difference in outcome between treatment modalities was analysed.
RESULTS: Of the 40 children studied, all had fever of 38.5 °C for more than 7 d. Splenomegaly was noted in 25 children at admission, but eventually occurred in all the patients. All children had bicytopenia. Mean laboratory values were as follows- neutrophil count 3,400/cu.mm, hemoglobin 8.75 g/dl, platelet count 84,000/cu.mm, fasting triglycerides 358 mg/dl, ferritin 8,139 mg/dl and fibrinogen 137 mg/dl. All children had evidence of hemophagocytosis in bone marrow smear. Good outcome was seen in 19/22 children treated with IVIG therapy (Group 1) vs. 10/12 children treated with HLH-2004 protocol with etoposide, cyclosporine and Dexamethasone (Group 2), P = 1.00. Good outcome was seen in 4/6 children treated with IVIG therapy followed by HLH-2004 protocol (Group 3). Serum ferritin levels of more than 3,000 mg/dl were present in 13 children. In this group, good outcome was seen in 7/8 patients treated with IVIG vs. 4/5 treated with the HLH-2004 protocol (P = 1.00).
CONCLUSIONS: IVIG and HLH-2004 protocol may be equally effective in the management of HLH. IVIG may be a preferable initial regimen, to avoid the risk of secondary malignancy associated with etoposide.