title: [Hemophagocytic syndromes and primary immunodeficiencies].
abstract: Lymphohistiocytic activation syndromes are characterized by activation and proliferation of T cells and macrophages usually reflecting an inappropriate response of the host to an infection. The clinical manifestations are often alarming symptoms suggestive of severe sepsis. Most patients have a known underlying disease (hemopathy, lupus, Still's disease, HIV infection, bone marrow or organ transplantation). In the few cases that occur apparently without any risk factors, investigations should be done to look for a predisposing inherited disease such as familial lymphohistiocytosis or Purtilo syndrome in boys. The treatment relies on immunosuppressive agents followed by bone marrow transplantation, which can provide a definitive cure in genetics forms.
authors.affiliation: Unité d'hématologie et oncologie pédiatrique, hôpital NORD, CHU de Saint-Etienne, 42055 Saint-Etienne, France. j.louis.stephan@chu-st-etienne.fr
authors.forename: J L
authors.initials: JL
authors.lastname: Stéphan
mesh_terms: D002648:Child; D015616:Histiocytosis, Non-Langerhans-Cell / Q000276:immunology* / Q000628:therapy; D006706:Homeostasis; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology* / Q000628:therapy; D008214:Lymphocytes / Q000276:immunology; D008232:Lymphoproliferative Disorders / Q000276:immunology; D010859:Pigmentation Disorders / Q000276:immunology; D014780:Viruses / Q000276:immunology