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Familial hemophagocytic lymphohistiocytosis

Synonym(s) Familial HLH
Prevalence Unknown
Inheritance Autosomal recessive
Age of onset Infancy
  • D76.1
  • C0272199
MeSH -
  • 10070904


Familial Hemophagocytic lymphohistiocytosis (FHL) is a rare primary immunodeficiency characterized by a macrophage activation syndrome (see this term) with an onset usually occurring within a few months or less common several years after birth.

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Detailed information

Review article
Clinical genetics review
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