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Hemophagocytic lymphohistiocytosis in children
Affiliations- Katedra i Klinika Pediatrii, Hematologii i Onkologii, Warszawski Uniwersytet Medyczny, Kierownik kliniki: Prof. dr. hab. Michał Matysiak, Warszawa, Polska
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Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a heterogenic syndrome characterized by an acute, life-threatening inflammation due to a highly stimulated but ineffective immune response. Depending on the etiology, HLH is divided into primary (genetic) and secondary (acquired) forms. Primary HLH can be divided into familial HLH and HLH associated with other genetic disorders. Secondary HLH usually occurs in the context of a severe infection, rheumatic disease, or malignancy. HLH in children is a rare condition characterized by nonspecific symptoms and poor prognosis. Novel diagnostic tools and therapeutic methods give hope to improve the survival of the patients.
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a heterogenic syndrome characterized by an acute, life-threatening inflammation due to a highly stimulated but ineffective immune response. Depending on the etiology, HLH is divided into primary (genetic) and secondary (acquired) forms. Primary HLH can be divided into familial HLH and HLH associated with other genetic disorders. Secondary HLH usually occurs in the context of a severe infection, rheumatic disease, or malignancy. HLH in children is a rare condition characterized by nonspecific symptoms and poor prognosis. Novel diagnostic tools and therapeutic methods give hope to improve the survival of the patients.
Keywords
Hemophagocytic lymphohistiocytosis; HLH; Cytotoxicity; Degranulation; NK cells; Perforin
About this articleTitle
Hemophagocytic lymphohistiocytosis in children
Journal
Issue
Pages
233-241
Published online
2016-10-01
Page views
110
Article views/downloads
1377
DOI
10.1016/j.achaem.2016.10.001
Bibliographic record
Acta Haematol Pol 2016;47(4):233-241.
Keywords
Hemophagocytic lymphohistiocytosis
HLH
Cytotoxicity
Degranulation
NK cells
Perforin
Authors
Magdalena Wołowiec
Iwona Malinowska