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[Treatment of ITP and AIHA in CVID: A systematic literature review].

Authors
  • Lacombe, V1
  • Lozac'h, P1
  • Orvain, C2
  • Lavigne, C3
  • Miot, C4
  • Pellier, I5
  • Urbanski, G6
  • 1 Service de médecine interne et maladies vasculaires, CHU d'Angers, 4, rue Larrey, 49000 Angers, France. , (France)
  • 2 Service des maladies du sang, CHU d'Angers, 4, rue Larrey, 49000 Angers, France. , (France)
  • 3 Service de médecine interne et maladies vasculaires, CHU d'Angers, 4, rue Larrey, 49000 Angers, France; Centre de référence des déficits immunitaires primitifs CEREDIH, CHU d'Angers, site constitutif Angers, 4, rue Larrey, 49000 Angers, France. , (France)
  • 4 Centre de référence des déficits immunitaires primitifs CEREDIH, CHU d'Angers, site constitutif Angers, 4, rue Larrey, 49000 Angers, France; Service d'immunologie-hématologie et oncologie pédiatriques, CHU d'Angers, 4, rue Larrey, 49000 Angers, France; Laboratoire d'immunologie et allergologie, CHU d'Angers, 4, rue Larrey, 49000 Angers, France. , (France)
  • 5 Centre de référence des déficits immunitaires primitifs CEREDIH, CHU d'Angers, site constitutif Angers, 4, rue Larrey, 49000 Angers, France; Service d'immunologie-hématologie et oncologie pédiatriques, CHU d'Angers, 4, rue Larrey, 49000 Angers, France. , (France)
  • 6 Service de médecine interne et maladies vasculaires, CHU d'Angers, 4, rue Larrey, 49000 Angers, France; Centre de référence des déficits immunitaires primitifs CEREDIH, CHU d'Angers, site constitutif Angers, 4, rue Larrey, 49000 Angers, France. Electronic address: [email protected] , (France)
Type
Published Article
Journal
La Revue de medecine interne
Publication Date
Aug 01, 2019
Volume
40
Issue
8
Pages
491–500
Identifiers
DOI: 10.1016/j.revmed.2019.02.006
PMID: 31101329
Source
Medline
Keywords
Language
French
License
Unknown

Abstract

Ten to 15% of common variable immunodeficiencies (CVID) develop auto-immune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Treatment is based on immunosuppressants, which produce blocking effects in the CVID. Our objective was to assess their risk-benefit ratio in these immunocompromised patients. We identified 17 articles detailing the treatment of AIHA and/or ITP in patients suffering from CVID through a systematic review of the MEDLINE database. The increased infectious risk with corticosteroids does not call into question their place in the first line of treatment of ITP and AIHA in CVID. High-doses immunoglobulin therapy remain reserved for ITP with a high risk of bleeding. In second-line treatment, rituximab appears to be effective, with a lower infectious risk than the splenectomy. Immunosuppressants (azathioprine, methotrexate, mycophenolate, cyclophosphamide, vincristine, ciclosporine) are moderately effective and often lead to severe infections, meaning that their use is justified only in resistant cases and steroid-sparing. Dapsone, danazol and anti-D immunoglobulins have an unfavorable risk-benefit ratio. The place of TPO receptor agonists is still to be defined. The establishment of immunoglobulin replacement in the place of immunosuppressants (except for short-term corticotherapy) or splenectomy appears to be essential to limit the risk of infections, including in the absence of previous infections. The presence of CVID does not mean that it is necessary to give up on corticosteroids as a first-line treatment and rituximab as a second-line treatment for AIHA and ITP, but it should be in addition to immunoglobulin replacement. A splenectomy should be reserved as a third-line treatment. Copyright © 2019 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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