Document Type : Original Article
Authors
- Samira Aggoune 1
- Hachemi Maouche 2
- Linda Chikhi 3
- Feriel Mekimene 3
- Samir Benmamar 3
- Despina Moshous 4
- Capucine Picard 5
- Genevieve De Saint Basile 5
- Jean Donadieu 6
- Guy Leverger 7
1 Ph.D, Departement of Pediatric’s, University Teaching Hospital, Belfort, Algier’s, Algeria
2 Departement of Pediatric’s, University Teaching Hospital, Belfort, Algier’s, Algeria.
3 Departement of Hemobiology University Teaching Hospital Mustapha Bacha, Algier’s, Algeria.
4 Center for the Study of Immune Deficits Tour, University Teaching Hospital Necker, Paris, France.
5 Center of Normal and Pathological Homeostasis of the Immune System Laboratory, University Teaching Hospital Necker, Paris, France.
6 Unit of Pediatric Immunology, Hematology and Rheumatology (UIHR); University Teaching Hospital Necker, Paris, France
7 Pediatric Hemato-Oncology Department, University Teaching Hospital Trousseau Paris, France.
Abstract
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.
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