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
DOI:
https://doi.org/10.18203/2349-3291.ijcp20192773Keywords:
Ferritn, Hemophagocytic lymphohistiocytosis, Injectable methyprednisolone, Macrophage activation syndrome, Oral prednisolone, TriglyceridesAbstract
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.
References
Ladisch S. Histiocytosis syndromes of childhood. In: Nelson Textbook of Pediatrics. 1st Ed. South Asia Edition: Elsevier; 2015: 2484-2489.
Dua V, Yadav SP, Sachdeva A. Hemophagocytic lymphohistiocytosis. In: Advances in Pediatrics. 2nd Ed. Jaypee; 2012: 612-618.
George MR, Hemophagocytic lymphocytosis: review of etiologies and management. J Blood Med. 2014;5:69-86.
Esteban YM, Jong JL, Tesher MS. An overview of hemophagocytic lymphohistiocytosis. Pediatric Ann. 2017;46(8):e309-13.
Rosado FGN, Kim AS, Hemophagocytic lymphohis-tiocytosis an update on diagnosis and pathogenesis.Am J Clin Pathol. 2013:139:713-27.
Ishii E. Hemophagocytic lymphohistiocytosis in children: pathogenesis and treatment. Frontiers Pedia. 2016;13(4):47.
Ravelli A, Grom AA, Behrens EM, Cron RQ. Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment. Genes Immunity. 2012;13:289-98.
Contino A, Trombatore G, Timeus F. Hemophagocytic lymphohistiocytosis in pediatric patients: a review. J Blood Dis Symp Treat. 2018;2(1):1-5.
Sasidharan PK, Priyadarshini B. Hemophagocytic lymphohistiocytosis: case history and review of literature. J Thrombo Cir. 2016;2(2):1-6.
Chatura KR, Mohan B. Liver biopsy to the aid in diagnosis of pediatric hemophagocytic lymphohistiocytosis: a case report. J Med Radiol Pathol Surg. 2017;4(3):6-9.
D’Souza E, Cheriathu JJ, Shamseldeen M. Rare case of hemophagocytic lymphohistiocytosis: a case report. GMJ ASM. 2012;1(S1):S194-S19.
Trottestam H, Horne A, Aricò M, Egeler RM, Filipovich AH, Gadner H, et al. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood. 2011;118(17):4577-84.
Henter JI, Horne SA, Arico M, Egeler RM, Elinder G, Filipovich AH, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. 2002;100(7):2367-73.
Wang Y, Huang W, Hu L, Cen X, Li L, Wang J, et al. Multicenter study of combination DEP regimen as a salvage therapy for adult refractory hemophagocytic lymphohistiocytosis. Blood. 2015;126(19): 2186-92.
Nawata T, Kubo M, Shiragami K, Nakamura Y, Yano M. Successful treatment of hemophagocytic lymphohistiocytosis associated with lupus nephritis by using mycophenolate mofetil. Case Rep Rheumatol. 2016:1-3.
Naoi T, Morita M, Kawakami T, Fujimoto S. Hemophagocytic lymphohistiocytosis associated with scrub typhus: systematic review and comparison between pediatric and adult cases. Trop Med Inf Dis. 2018:3(1).
Jordan MB, Allen CE, Weizman S, Filipouich AH, Milian KL. How I treat HLH. Blood. 2011;118:4041-52.
Giri PP, Khemka P, Roy S, Bhattyacharya S. Hemophagocytic lymphohistiocytosis-a rare complication of hepatitis a virus infection. Arch Pediatr Inf Dis. 2015;3(3):e21150.
Ramachandran B, Balasubramanium S, Abhishek N, Ravikumar KG, Ramanan AV. Profile of hemophagocytic lymphohistiocytosis in children in a tertiary care hospital in India. Indian Pedia. 2011;48:31-5.
Demirkol D, Yildizdas D, Bayrakci B, Karapinar B, Kendirli T, Koroglu TF, et al. Hyperferritinemia in critically ill child with secondary hemophagocytic lymphohistiocytosis/sepsis/multi organ dysfunction syndrome/macrophage activation syndrome: what is the treatment?. Crit Care. 2012;16(2):R52.
