Article
Genetics and pathophysiology of haemophagocytic lymphohistiocytosis.
Acta paediatrica (Oslo, Norway : 1992) - 1 Nov 2021
Meeths Marie, Bryceson Yenan T
Abstract excerpt
Haemophagocytic lymphohistiocytosis (HLH) represents a life-threatening hyperinflammatory syndrome. Familial studies have established autosomal and X-linked recessive causes of HLH, highlighting a pivotal role for lymphocyte cytotoxicity in the control of certain virus infections and immunoregulation. Recently, a more complex etiological framework has emerged, linking HLH predisposition to variants in genes...
Topics
- Genetic Predisposition to Disease
- Humans
- Lymphohistiocytosis, Hemophagocytic
- Virus Diseases
