A hidden enemy: Understanding the hemophagocytic syndrome in children under five years of age in a high-complexity institution in southwestern Colombia
Abstract
Introduction. Hemophagocytic syndrome is an under-recognized condition with high mortality in the pediatric population. It is characterized by excessive activation of immune cells and cytokine release, leading to persistent inflammation. Hemophagocytic syndrome can be primary or secondary and associated with different triggers.
Objective. To describe 12 clinical cases of children under five years of age with hemophagocytic syndrome in a high-complexity institution in southwestern Colombia.
Materials and methods. We present a retrospective series of 12 cases of hemophagocytic syndrome in children under five years of age treated at a high-complexity institution in Colombia between 2019 and 2022.
Results. The median age of the patients was one year and 7 were male. Fever and splenomegaly were the most common clinical manifestations observed in 11 of the
patients. The predominant laboratory findings included hyperferritinemia (n = 11), hypertriglyceridemia (n = 10), bicytopenia (n = 6), and pancytopenia (n = 2). Eleven cases had elevated lactate dehydrogenase levels. Genetic studies were conducted in 7 patients. Regarding treatment, the full HLH-2004 protocol was administered to 5 cases, while 3 underwent hematopoietic stem cell transplantation. Three patients died.
Conclusion. We highlight the complexity of the hemophagocytic syndrome, especially in children under five years old, because the low prevalence and non-specific clinical presentation of the disease contribute to its underdiagnosis. Emphasis is placed on identifying triggers, performing genetic evaluation for accurate and early diagnosis, adopting a multidisciplinary approach, and considering early hematopoietic stem cell transplantation to improve morbidity and mortality outcomes.
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References
Malinowska I, Machaczka M, Popko K, Siwicka A, Salamonowicz M, Nasiłowska-Adamska B. Hemophagocytic syndrome in children and adults. Arch Immunol Ther Exp. 2014;62:385-94. https://doi.org/10.1007/s00005-014-0274-1
Henderson L, Cron R. Macrophage activation syndrome and secondary hemophagocytic lymphohistiocytosis in childhood inflammatory disorders: Diagnosis and management. Pediatri Drugs. 2020;22:29-44. https://doi.org/10.1007/s40272-019-00367-1
Griffin G, Shenoi S, Hughes G. Hemophagocytic lymphohistiocytosis: An update on pathogenesis, diagnosis, and therapy. Best Pract Res Clin Rheumatol. 2020;34:101515. https://doi.org/10.1016/j.berh.2020.101515
Hernández P, Díaz C, Laureiro J, Madrid O, Martín E, Lumbreras C. Hemophagocytic lymphohistiocytosis: Analysis of 18 cases. Med Clin (Barc). 2016;147:495-8. https://doi.org/10.1016/j.medcli.2016.07.031
Bergsten E, Horne A, Aricó M, Astigarraga I, Egeler R, Filipovich A, et al. Confirmed efficacy of etoposide and dexamethasone in HLH treatment: Long-term results of the cooperative HLH-2004 study. Blood. 2017;130:2728-38. https://doi.org/10.1182/blood-2017-06-788349
Jovanovic A, Kuzmanovic M, Kravljanac R, Micic D, Jovic M, Gazikalovic S, et al. Central nervous system involvement in hemophagocytic lymphohistiocytosis: A single-center experience. Pediatri Neurol. 2014;50:233-7. https://doi.org/10.1016/j.pediatrneurol.2013.10.014
Henter J, Horne AC, Aricó M, Egeler R, Filipovich A, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007;48:124-31. https://doi.org/10.1002/pbc.21039
Janka G, Lehmberg K. Hemophagocytic syndromes - An update. Blood Rev. 2014;28:135-42. https://doi.org/10.1016/j.blre.2014.03.002
George M. Hemophagocytic lymphohistiocytosis: Review of etiologies and management. J Blood Med. 2014;69-86. https://doi.org/10.2147/JBM.S46255
Madkaikar M, Shabrish S, Desai M. Current updates on classification, diagnosis and treatment of hemophagocytic lymphohistiocytosis (HLH). Indian J Pediatr. 2016;83:434-43. https://doi.org/10.1007/s12098-016-2037-y
Alvi S, Naqvi A. Advances in the diagnosis and management of haemophagocytic lymphohistiocytosis: A review of literature. Lympho Sign J. 2018;5:1-15. https://doi.org/10.14785/lymphosign-2017-0010
Astigarraga I, González L, Allende L, Alsina L. Síndromes hemofagocíticos: la importancia del diagnóstico y tratamiento precoces. An Pediatr (Barc). 2018;89:124.e1-124.e8. https://doi.org/10.1016/j.anpedi.2018.05.003
Ehl S, Astigarraga I, von Bahr Greenwood T, Hines M, Horne A, Ishii E, et al. Recommendations for the use of etoposide-based therapy and bone marrow transplantation for the treatment of HLH: Consensus statements by the HLH steering committee of the histiocyte society. J Allergy Clin Immunol Pract. 2018;6:1508–17. https://doi.org/10.1016/j.jaip.2018.05.031
Chinn I, Eckstein O, Peckham-Gregory E, Goldberg B, Forbes L, Nicholas S, et al. Genetic and mechanistic diversity in pediatric hemophagocytic lymphohistiocytosis. Blood. 2018;132:89-100. https://doi.org/10.1182/blood-2017-11-814244
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