open access

Vol 47, No 4 (2016)
Praca poglądowa / Review
Submitted: 2016-06-16
Published online: 2016-10-01
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Hemophagocytic lymphohistiocytosis in children

Magdalena Wołowiec1, Iwona Malinowska
DOI: 10.1016/j.achaem.2016.10.001
·
Acta Haematol Pol 2016;47(4):233-241.
Affiliations
  1. Katedra i Klinika Pediatrii, Hematologii i Onkologii, Warszawski Uniwersytet Medyczny, Kierownik kliniki: Prof. dr. hab. Michał Matysiak, Warszawa, Polska

open access

Vol 47, No 4 (2016)
Praca poglądowa / Review
Submitted: 2016-06-16
Published online: 2016-10-01

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.

Get Citation

Keywords

Hemophagocytic lymphohistiocytosis; HLH; Cytotoxicity; Degranulation; NK cells; Perforin

About this article
Title

Hemophagocytic lymphohistiocytosis in children

Journal

Acta Haematologica Polonica

Issue

Vol 47, No 4 (2016)

Pages

233-241

Published online

2016-10-01

Page views

178

Article views/downloads

1830

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

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