Document Type : Original Article
Ph.D, Departement of Pediatric’s, University Teaching Hospital, Belfort, Algier’s, Algeria
Departement of Pediatric’s, University Teaching Hospital, Belfort, Algier’s, Algeria.
Departement of Hemobiology University Teaching Hospital Mustapha Bacha, Algier’s, Algeria.
Center for the Study of Immune Deficits Tour, University Teaching Hospital Necker, Paris, France.
Center of Normal and Pathological Homeostasis of the Immune System Laboratory, University Teaching Hospital Necker, Paris, France.
Unit of Pediatric Immunology, Hematology and Rheumatology (UIHR); University Teaching Hospital Necker, Paris, France
Pediatric Hemato-Oncology Department, University Teaching Hospital Trousseau Paris, France.
Familial haemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive disorder of immune dysregulation associated with uncontrolled T cell and macrophage activation and hypercytokinaemia. HLH affects primarily pediatric population, mainly infants of less than 3 months of age. It is important to have a high index of suspicion for diagnosis since an early diagnosis is crucial to decrease the significant mortality associated the disease. This article gives a case historie and review the varied clinical presentations, pathophysiology, prognosis and treatment of the entity.