Neurological Involvement in Childhood Evans Syndrome View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2019-02

AUTHORS

Thomas Pincez, Bénédicte Neven, Hubert Ducou Le Pointe, Pascale Varlet, Helder Fernandes, Albane Gareton, Guy Leverger, Thierry Leblanc, Hervé Chambost, Gérard Michel, Marlène Pasquet, Frédéric Millot, Olivier Hermine, Alexis Mathian, Marie Hully, Hélène Zephir, Mohamed Hamidou, Jean-Marc Durand, Yves Perel, Judith Landman-Parker, Fréderic Rieux-Laucat, Nathalie Aladjidi

ABSTRACT

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. More... »

PAGES

171-181

References to SciGraph publications

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s10875-019-0594-3

DOI

http://dx.doi.org/10.1007/s10875-019-0594-3

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1111604120

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/30671780


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