Lymphohistiocytosis, Hemophagocytic

Known as: Hemophagocytic lymphohistiocytosis, Hemophagocytic Syndromes, Hemophagocytic Syndrome 
A rare but potentially life-threatening disorder characterized by the proliferation of histiocytes and macrophages and phagocytosis of red blood… (More)
National Institutes of Health

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21 relations
Epstein-Barr Virus InfectionsHPLH1 geneHemophagocytic SyndromeHistiocytosis haematophagic
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Narrower (7)

Familial Hemophagocytic LymphocytosisGRISCELLI SYNDROME, TYPE 2HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS, FAMILIAL, 2HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS, FAMILIAL, 3
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Broader (1)

Histiocytosis, Non-Langerhans-Cell

Papers overview

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Highly Cited
2007
Highly Cited
2007
HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.
In HLH-94, the first prospective international treatment study for hemophagocytic lymphohistiocytosis (HLH), diagnosis was based… (More)
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Review
2007
Review
2007
Hemophagocytic syndromes.
Hemophagocytic syndromes (hemophagocytic lymphohistiocytosis, HLH) represent a severe hyperinflammatory condition with the… (More)
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Review
2005
Review
2005
Familial and acquired hemophagocytic lymphohistiocytosis.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening condition of severe hyperinflammation caused by the uncontrolled… (More)
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Highly Cited
2005
Highly Cited
2005
Linkage of familial hemophagocytic lymphohistiocytosis (FHL) type-4 to chromosome 6q24 and identification of mutations in syntaxin 11.
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive disorder characterized by hyperactive phagocytes… (More)
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Highly Cited
2004
Highly Cited
2004
An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder.
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder with familial and acquired forms. The familial form is associated… (More)
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Highly Cited
2003
Highly Cited
2003
Munc13-4 Is Essential for Cytolytic Granules Fusion and Is Mutated in a Form of Familial Hemophagocytic Lymphohistiocytosis (FHL3)
Secretion of cytolytic granules content at the immunological synapse is a highly regulated process essential for lymphocyte… (More)
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Highly Cited
2002
Highly Cited
2002
Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation.
Hemophagocytic lymphohistiocytosis (HLH) comprises familial (primary) hemophagocytic lymphohistiocytosis (FHL) and secondary HLH… (More)
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Highly Cited
1999
Highly Cited
1999
Perforin gene defects in familial hemophagocytic lymphohistiocytosis.
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, rapidly fatal, autosomal recessive immune disorder characterized by… (More)
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Review
1998
Review
1998
Familial hemophagocytic lymphohistiocytosis. Primary hemophagocytic lymphohistiocytosis.
Hemophagocytic lymphohistiocytosis represents a spectrum of pathogenetically different diseases including the rapidly fatal… (More)
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Review
1983
Review
1983
Familial hemophagocytic lymphohistiocytosis
Familial hemophagocytic lymphohistiocytosis (FHL) is probably a genetically transmitted disease affecting infants and very young… (More)
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