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Familial haemophagocytic lymphohistiocytosis: advances in the genetic basis, diagnosis and management

Familial haemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive disorder of immune dysregulation associated with uncontrolled T cell and macrophage activation and hypercytokinaemia. The incidence of FHL is 0·12/100·000 children born per year, with a male to female ratio of 1:1. The...

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Detalhes bibliográficos
Main Authors: Gholam, C, Grigoriadou, S, Gilmour, K C, Gaspar, H B
Formato: Artigo
Idioma:Inglês
Publicado em: Blackwell Science Inc 2011
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3048610/
https://ncbi.nlm.nih.gov/pubmed/21303357
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1111/j.1365-2249.2010.04302.x
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