title: Neuroradiologic findings and follow-up with magnetic resonance imaging of the genetic forms of haemophagocytic lymphohistiocytosis with CNS involvement.
abstract: Haemophagocytic lymphohistiocytosis (HLH) is a rare hyperinflammatory syndrome caused by deficient down-regulation of the immune response. Presence of central nervous system (CNS) involvement at diagnosis is a poor prognostic sign, and should be carefully investigated. Herein, we describe the neuroradiological findings, clinical data, and treatment outcome in 12 patients with genetic HLH and CNS complications. Neuroimaging was important in identifying CNS involvement, monitoring treatment responses, and detecting treatment complications.
authors: Neuroradiology Department, G. Gaslini Children's Hospital, Genoa, Italy.
authors: Ines
authors: I
authors: Rego
authors: Mariasavina
authors: M
authors: Severino
authors: Concetta
authors: C
authors: Micalizzi
authors: Maura
authors: M
authors: Faraci
authors: Daniela
authors: D
authors: Pende
authors: Carlo
authors: C
authors: Dufour
authors: Maurizio
authors: M
authors: Aricò
authors: Andrea
authors: A
authors: Rossi
mesh_terms: D000293:Adolescent; D001921:Brain / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications / Q000473:pathology* / Q000628:therapy; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D059906:Neuroimaging; D016896:Treatment Outcome