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.

A Case of Steroid Glaucoma in a Child Treated with Systemic Steroid as Graft-Versus-Host Disease

PURPOSE: To report a case of steroid-induced glaucoma in a child who was treated with systemic steroids for a long period due to graft-versus-host disease. CASE SUMMARY: A 10-year-old male was referred to our ophthalmologic clinic for examination of papilledema due to persistent headache and nausea. He was diagnosed as aplastic anemia 8 years prior and took approximately 4,000 mg of oral prednisolone for 8 years from April 2007 to April 2015 for treatment of lung graft-versus-host disease after hematopoietic stem cell transplantation. His best corrected visual acuity was 0.8 (decimal) in the right eye, 0.5 in the left eye and intraocular pressure (IOP) measured using a Goldmann applanation tonometer was 42 mm Hg in the right eye and 43 mm Hg in the left eye. His cup-to-disc ratio was 0.8 in the right eye and 0.7 in the left eye. Additionally, superior and inferior neuroretinal rim thinning was present in both eyes. Despite using IOP-lowering agents, IOP was not controlled. However, after trabeculectomy with mitomycin C in both eyes, IOP became normalized. CONCLUSIONS: In cases of pediatric patients treated with systemic steroids for a long period of time, regular observation is necessary to prevent IOP elevation and steroid-induced glaucoma.

Long-term Follow-up of an Evans Syndrome with Chronic, Refractory Course: Response to 5 Different Regimens / 대한소아혈액종양학회지

Evans syndrome is the combination of direct Coombs' positive hemolytic anemia and immune thrombocytopenic purpura, in the absence of a known underlying etiology. Being reported rarely in pediatric patients, the syndrome is characterized by periods of remission and exacerbation with viable responses to therapy. Management of the disease remains a challenge despite a variety of therapeutic trials. We experienced a 11-years old male patient of Evans syndrome who was initially presented as having an autoimmune hemolytic anemia 17 months before. Over the 5 years of follow-up, he had a chronic, relapsing courses, showing partial responses to a variety of therapeutic trials, including IVIG, oral prednisolone, methylprednisolone pulse therapy, cyclosporine A and vincristine. A brief review of the literature ensues with the case report.

Eruptive Neonatal Hemangiomatosis

Eruptive neonatal hemangiomatosis is an uncommon disorder, which is divided into two types according to the extent of involvement: Benign type with cutaneous hemangiomatosis only and diffuse type with widespread hemangiomas of skin and viscera. The organs commonly affected are the gastrointestinal tract, brain, liver and lung. The Diffuse type is often fatal. We herein report a case of eruptive neonatal hemangtomatosis with cutaneous and hepatic involvement. The size of the hepatic arteriovenous malformation was markedly decreased after 2 months' therapy with oral prednisolone.