Neurological Involvement in Childhood Evans Syndrome - Sorbonne Université Accéder directement au contenu
Article Dans Une Revue Journal of Clinical Immunology Année : 2019

Neurological Involvement in Childhood Evans Syndrome

Pascale Varlet
Helder Fernandes
Alexis Mathian
  • Fonction : Auteur
  • PersonId : 1040423
Yves Perel
  • Fonction : Auteur
Nathalie Aladjidi
  • Fonction : Auteur

Résumé

PURPOSE: Immune thrombocytopenic purpura (ITP) and autoimmune hemolytic anemia (AIHA) are associated in the definition of Evans syndrome (ES). The occurrence of neurological involvement in this population is poorly described and suggests an underlying primary immunodeficiency (PID). We aimed to describe the clinical manifestations, evolution, and PID profiles of these patients. METHODS: OBS'CEREVANCE is a French, nationwide prospective cohort that includes children with chronic ITP, AIHA, and ES. Patients with a neurological involvement were described. Centralized radiological and pathological reviews and genetic analyses were performed. RESULTS: On October 2016, eight patients (7/181 ES, 1/371 AIHA, and 0/615 ITP) were identified, all male, with a median age (range) at cytopenia onset of 11.5 years (1.6-15.8). Neurological symptoms appeared with a median delay of 6 years (2.5-18) after cytopenia and were polymorphic: seizures (n = 4), cranial nerve palsy (n = 2), Brown-Sequard syndrome (n = 2), intracranial pressure (n = 2), vertigo (n = 1), and/or sensory neuropathy (n = 1). Magnetic resonance imaging (MRI) showed inflammatory lesions, confirmed by pathology for five patients with macrophagic or lymphoplasmocytic infiltrates. All patients had other relevant immunopathological manifestations: pulmonary nodules (n = 6), lymphoproliferation (n = 4), abnormal immunophenotype (n = 8), and hypogammaglobulinemia (n = 7). Treatment consisted of steroids that improved symptomatology and MRI. Five patients relapsed and three had an asymptomatic radiological progression. A PID was identified in 3/8 patients: 22q11.2 microdeletion (n = 1) and CTLA deficiency (n = 2). CONCLUSION: Neurological involvement is a rare and severe late event in the course of childhood ES, which can reveal an underlying PID. Imaging and pathology examination highlight a causative immune dysregulation that may guide targeted therapeutic strategies.
Fichier principal
Vignette du fichier
Pincez et al. - 2019 - Neurological Involvement in Childhood Evans Syndro.pdf (7.53 Mo) Télécharger le fichier
Origine : Fichiers produits par l'(les) auteur(s)
Loading...

Dates et versions

hal-02366149 , version 1 (15-11-2019)

Identifiants

Citer

Thomas Pincez, Bénédicte Neven, Hubert Ducou Le Pointe, Pascale Varlet, Helder Fernandes, et al.. Neurological Involvement in Childhood Evans Syndrome. Journal of Clinical Immunology, 2019, 39 (2), pp.171-181. ⟨10.1007/s10875-019-0594-3⟩. ⟨hal-02366149⟩
286 Consultations
394 Téléchargements

Altmetric

Partager

Gmail Facebook X LinkedIn More