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A retrospective longitudinal study on the effect of ultra short course of steroid therapy on clinical and hematologic parameters of secondary hemophagocytic lymphohistiocytosis in children

Background: The aim was to study the effect of ultra-short course of injectable steroid followed by oral steroid therapy for cumulative 14 days on clinical and hematologic parameters of secondary hemophagocytic lymphohistiocytosis in children.Method: A retrospective longitudinal study was conducted by collecting data from case records. Cases diagnosed with secondary hemophagocytic lymphohistiocytosis had been included. The cases with malignancy were excluded. Remaining cases had been given injectable methyprednisolone (30 mg/kg/day) for three days followed by oral prednisolone (1 mg/kg/day) for 11 days. The time  to cessation of fever and organomegaly were noted. The changes in mean hematologic parameters, ferritin and triglycerides were noted at the time of suspicion of HLH or MAS, after therapy and on follow up.Results: About 96% of children were afebrile within five days of therapy. There was regression of hepatospleenomegaly in all 100% surviving children by day seven of therapy. Improvement in hemoglobin (mean value 8.1 mg/dl to 8.7 mg/dl) and platelet count (mean value 0.89 lakh to 1.47 lakh) was seen by day seven of therapy. Fall in serum ferritn (mean value 1419 ng/ml to 298 ng/ml) and serum triglycerides (mean value 307 mg/dl to 176 mg/dl) was seen at one-month follow-up. 96% survival was observed. None of the survivors had any recurrence at 6 months follow up.Conclusion: Ultra short course of injectable methyprednisolone for 3 days followed by oral prednisolone for 11 days was successful in 95% survival in our study. This cost-effective regimen, with use of less toxic drugs leading to a shorter hospital stay maybe helpful in resource limited settings.