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• W2945756537 abstract "Dix à 15 % des déficits immunitaires communs variables (DICV) se compliquent d’anémie hémolytique auto-immune (AHAI) et de purpura thrombopénique immunologique (PTI). Leur traitement repose sur des immunosuppresseurs, générant certaines réticences dans le DICV. Notre objectif était d’évaluer leur rapport bénéfices-risques chez ces patients immunodéprimés. Nous avons réalisé une revue systématique de la littérature (base bibliographique MEDLINE), identifiant 17 articles détaillant le traitement d’AHAI et/ou de PTI chez les patients atteints de DICV. Le risque infectieux majoré sous corticoïdes ne remet pas en question leur place en première ligne dans le PTI et l’AHAI du DICV. Les immunoglobulines à forte dose restent à réserver aux PTI à risque hémorragique important. En deuxième ligne, le rituximab semble efficace avec un risque infectieux moindre que la splénectomie. Les immunosuppresseurs (azathioprine, méthotrexate, mycophénolate, cyclophosphamide, vincristine, ciclosporine) sont d’efficacité inconstante et souvent compliqués d’infections sévères, ne se justifiant que pour les cas réfractaires et en cortico-épargne. Dapsone, danazol et immunoglobulines anti-D ont un rapport bénéfices-risques défavorable. La place des agonistes du récepteur de la thrombopoïétine reste à définir. L’instauration systématique d’immunoglobulines substitutives aux traitements à effet immunosuppresseurs (hors courte corticothérapie) ou à la splénectomie apparaît essentielle pour limiter le risque infectieux, y compris en l’absence d’infections préalables. La présence d’un DICV ne doit pas faire renoncer à la corticothérapie en première intention et au rituximab en seconde intention en traitement des AHAI et PTI, mais doit faire adjoindre une substitution en immunoglobulines. La splénectomie semble à réserver en troisième intention. 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." @default.
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• W2945756537 date "2019-08-01" @default.
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• W2945756537 title "Traitement du PTI et de l’AHAI au cours du DICV : revue systématique de la littérature" @default.
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• W2945756537 doi "https://doi.org/10.1016/j.revmed.2019.02.006" @default.
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