Volume 3, Issue 1 (2-2019)                   GMJ Medicine 2019, 3(1): 95-98 | Back to browse issues page


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1- Departement of Pediatric’s, University Teaching Hospital, Belfort, Algier’s, Algeria
2- Departement of Hemobiology University Teaching Hospital Mustapha Bacha, Algier’s, Algeria
3- Center for the Study of Immune Deficits Tour, University Teaching Hospital Necker, Paris, France
4- Center of Normal and Pathological Homeostasis of the Immune System Laboratory, University Teaching Hospital Necker, Paris, France
5- Unit of Pediatric Immunology, Hematology and Rheumatology (UIHR); University Teaching Hospital Necker, Paris, France
6- Pediatric Hemato-Oncology Department, University Teaching Hospital Trousseau Paris, France
Abstract:   (1908 Views)
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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Type of Study: Clinical Case Report | Subject: Immunology
Received: 2019/03/15 | Accepted: 2019/05/25 | Published: 2019/05/30

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