Paediatric‐onset Evans syndrome: Breaking away from refractory immune thrombocytopenia

Author:

Aladjidi Nathalie12ORCID,Pincez Thomas13ORCID,Rieux‐Laucat Frédéric4,Nugent Diane5ORCID

Affiliation:

1. Centre de Référence National des Cytopénies Auto‐immunes de l'Enfant (CEREVANCE) Bordeaux France

2. Pediatric Hemato‐Immunology, CIC1401, INSERM CICP Bordeaux University Hospital Bordeaux France

3. Division of Pediatric Hematology‐Oncology, Department of Pediatrics, Charles‐Bruneau Cancer Center, Sainte‐Justine University Hospital Université de Montréal Québec Montréal Canada

4. Université Paris Cité, Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, Institut Imagine INSERM UMR 1163 Paris France

5. Division of Hematology, Childrens Hospital of ORange County (CHOC) University of California Irvine Irvine California USA

Abstract

SummarySince its first description by Evans in 1951, this syndrome has been linked to chronic immune thrombocytopenia with the concurrent or delayed onset of autoimmune haemolytic anaemia or neutropenia. For decades, the evolution of Evans syndrome (ES) has carried a poor prognosis and often resulted in chronic steroid exposure, multiple immune suppressing medications directed against T or B lymphocytes, and splenectomy. This paper presents a new view of ES based on recent advances in genomics which begin to classify patients based on their underlying molecular variants in previously described primary immune disorders. This has opened up new avenues of targeted therapy or bone marrow transplant at rather than broad long‐term immune suppression or splenectomy. Importantly, recent studies of the full lifespan of ES suggest that at least 80% of those paediatric patients will progress to various clinical or biological immunopathological manifestations with age despite the resolution of their cytopenias. Those patients merit long‐term follow‐up and monitoring in dedicated transition programs to improve outcome at the adult age.

Funder

Institut National de la Santé et de la Recherche Médicale

Agence Nationale de la Recherche

Fondation pour la Recherche Médicale

Publisher

Wiley

Subject

Hematology

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