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Isolated central nervous system familial hemophagocytic lymphohistiocytosis (fHLH) presenting as a mimic of demyelination in children.
- Source :
-
Multiple sclerosis (Houndmills, Basingstoke, England) [Mult Scler] 2022 Apr; Vol. 28 (4), pp. 669-675. Date of Electronic Publication: 2021 Oct 27. - Publication Year :
- 2022
-
Abstract
- Isolated central nervous system (CNS) presentations of haemophagocytic lymphohistiocytosis (HLH), traditionally a systemic inflammatory condition, have been reported in adults and children. We identified nine patients with a diagnosis of isolated CNS familial hemophagocytic lymphohistiocytosis (fHLH) with symptom onset <18 years of age, and one asymptomatic sibling. Children with atypical chronic/recurrent CNS inflammation should be considered for immunological and genetic panel testing for fHLH even in the absence of any systemic inflammatory features. Despite haematopoietic stem cell transplantation (HSCT) being a mainstay of treatment, treatment failure and high morbidity and mortality post-HSCT suggest that alternative immune therapies may be worth considering.
Details
- Language :
- English
- ISSN :
- 1477-0970
- Volume :
- 28
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Multiple sclerosis (Houndmills, Basingstoke, England)
- Publication Type :
- Academic Journal
- Accession number :
- 34704499
- Full Text :
- https://doi.org/10.1177/13524585211053565