Incidence and clinical presentation of primary hemophagocytic lymphohistiocytosis in Sweden

Background Primary hemophagocytic lymphohistiocytosis (HLH) represents a group of inherited hyperinflammatory immunodeficiencies, including familial HLH (FHL), Griscelli syndrome type 2 (GS2), and X‐linked lymphoproliferative syndrome (XLP). We previously reported an annual incidence of suspected pr...

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Bibliographic Details
Published in:Pediatric blood & cancer 2015-02, Vol.62 (2), p.346-352
Main Authors: Meeths, Marie, Horne, AnnaCarin, Sabel, Magnus, Bryceson, Yenan T., Henter, Jan-Inge
Format: Article
Language:English
Subjects:
Adolescent
Child
Child, Preschool
cytotoxic lymphocyte
Hematologi
Hematology
Hematopoietic Stem Cell Transplantation
hemophagocytic lymphohistiocytosis
Humans
incidence
Infant
Lymphohistiocytosis, Hemophagocytic - epidemiology
Lymphohistiocytosis, Hemophagocytic - mortality
Lymphohistiocytosis, Hemophagocytic - therapy
Oncology
Pediatrics
Pediatrik
primary immunodeficiency
Registries
Survival Rate
Sweden - epidemiology
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Summary:Background Primary hemophagocytic lymphohistiocytosis (HLH) represents a group of inherited hyperinflammatory immunodeficiencies, including familial HLH (FHL), Griscelli syndrome type 2 (GS2), and X‐linked lymphoproliferative syndrome (XLP). We previously reported an annual incidence of suspected primary HLH in Sweden 1971–1986 of 0.12 per 100,000 children. Here, we determined if the incidence had increased with concomitant awareness. Procedure Children
ISSN:1545-5009
1545-5017
1545-5017
DOI:10.1002/pbc.25308