Germany Journal

Document Type : Original Article

Authors

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.

Keywords