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Neuroradiologic findings and follow‐up with magnetic resonance imaging of the genetic forms of haemophagocytic lymphohistiocytosis with CNS involvement
Author(s) -
Rego Ines,
Severino Mariasavina,
Micalizzi Concetta,
Faraci Maura,
Pende Daniela,
Dufour Carlo,
Aricò Maurizio,
Rossi Andrea
Publication year - 2012
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.23405
Subject(s) - medicine , hemophagocytic lymphohistiocytosis , neuroimaging , magnetic resonance imaging , central nervous system , immune system , blood cancer , pediatrics , pathology , immunology , cancer , radiology , disease , psychiatry
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. Pediatr Blood Cancer 2012; 58: 810–814. © 2011 Wiley Periodicals, Inc.