Keywords
pediatrics
How to Cite
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening and generally underdiagnosed clinical entity. This syndrome describes patients with severe systemic hyperinflammation. The diagnosis is made on the basis of clinical, genetic and analytical criteria. Two main types of HLH are described: primary and secondary. Primary HLH is associated with genetic defects in: perforin (PRF), UNC13D, Syntaxin 11, Syntaxin BP2, molecules involved in the mechanism of cellular cytotoxicity. The sHLH are associated with infectious causes such as Epstein Barr virus (EBV), tuberculosis, brucellosis, leishmaniasis and SARS-CoV-2, rheumatologic (in this case it is defined as macrophage activation syndrome) and hemato-oncological causes.
References
Scott R, Robb A. Histiocytic medullary reticulosis. Lancet 1939; 234(6047):194-8.
Farquhar JW, Claireaux AE. Familial hemophagocytic reticulosis. Arch Dis Child 1952; 27: 519-525
Hadchouel M, Prieur AM, Griscelli C. Acute hemorrhagic, hepatic, and neurologic manifestations in juvenile rheumatoid arthritis: possible relationship to drugs or infection. J Pediatr. 1985; 106(4): 561-6.
Itziar Astigarraga et al, Síndromes hemofagocíticos: la importancia del diagnóstico y tratamiento precoces. An Pediatric (Barc). 2018; 89 (2):124 e1-124 e8.
Canna SW, Marsh RA. Pediatric hemophagocytic lymphohistiocytosis. Blood. 2020; 135:1332-43.
Sen ES, Steward CG, Ramanan AV. Diagnosing hemophagocytic syndrome. Arch Dis Child. 2017; 102:279-84.
Janka GE. Linfohistiocitosis hemofagocítica familiar. Eur J Pediatr 1983; 140:221–2306.
Janka GE, Schenider EM. Manejo moderno de niños con linfohistiocitosis hemofagocítica. Br J Haematol 2004, 124:4-14
Arceci RJ. When T cells and macrophages do not talk: the hemophagocytic syndromes. Curr Opin Hematol. 2008; 15:359-67.
Soy M, Atagündüz P, Atagündüz I, Sucak T. Hemophagocytic lymphohistiocytosis: a review inspired by the COVID-19 pandemic. Rheumatol Int. 2021; 41:7-18.
Histiocyte Society HLH-2004, Hemophagocytic Lymphohistiocytosis Study Group, Treatment Protocol of the Second International HLH Study 2004, Start of the Study: January 2004 Chairman: Jan-Inge Henter, M.D., Ph.D., Stockholm, Sweden
Imashuko S. Clinical features and treatment strategies of Epstein-Barr virus associated hemophagocytic lymphohistiocytosis. Crit Rev Oncol Hematol. 2002; 44:259-72.
Sen ES, Steward CG, Ramanan AV. Diagnosing haemophagocytic syndrome. Arch Dis Child. 2017; 102: 279-84.
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