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Genetic and Rare Diseases Information Center (GARD)

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Hemophagocytic lymphohistiocytosis

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Other Names for this Disease
  • Familial erythrophagocytic lymphohistiocytosis
  • Familial hemophagocytic lymphohistiocytosis
  • Familial histiocytic reticulosis
  • Hemophagocytic lymphohistiocytosis familial -1
  • HPLH1
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Is hemophagocytic lymphohistiocytosis inherited?

Hemophagocytic lymphohistiocytosis (HLH) may be familial (inherited) or secondary (acquired or reactive).

Familial hemophagocytic lymphohistiocytosis (FHL) is inherited in an autosomal recessive manner. This means that 2 mutated copies of the disease-causing gene (one inherited from each parent) are necessary to cause the condition. The parents of an individual with an autosomal recessive condition typically carry one mutated copy of the gene and are referred to as carriers. When 2 carriers of an autosomal recessive condition have children, each child has a 25% (1 in 4) risk to have the condition, a 50% (1 in 2) risk to be a carrier like each of the parents, and a 25% risk to not have the condition and not be a carrier.

Secondary HLH is not inherited and is usually associated with:

Secondary HLH is difficult to distinguish from familial HLH by clinical or histologic findings alone. Genetic testing is typically recommended even in HLH that is suspected to be acquired.[1]

Last updated: 11/26/2012

  1. Kejian Zhang, Alexandra H Filipovich, Judith Johnson, Rebecca A Marsh, and Joyce Villanueva. Hemophagocytic Lymphohistiocytosis, Familial. GeneReviews. September 7, 2010; Accessed 11/26/2012.