| Autor: |
Hbibi M; Department of hematology-Oncology pediatric SHOP, Hassan II University Hospital, Fez, Morocco., El Alaoui El Hanafi M; Department of hematology-Oncology pediatric SHOP, Ibn Rochd University Hospital, Casablanca, Morocco., Kasmi Z; Laboratory of clinical immunology, inflammation and allergy (LICIA), Faculty of medicine and pharmacy Casablanca, Morocco., Ouair H; Laboratory of clinical immunology, inflammation and allergy (LICIA), Faculty of medicine and pharmacy Casablanca. Morocco., Benmiloud S; Department of hematology-Oncology pediatric SHOP, Hassan II University Hospital, Fez, Morocco., Ailal F; Department of infectious diseases and clinical immunology pediatric, Ibn Rochd hospital Casablanca, Morocco., Hida M; Department of pediatric, Hassan II hospital Fez. Morocco., Bousfiha AA; Department of infectious diseases and clinical immunology pediatric, Ibn Rochd hospital Casablanca, Morocco. |
| Abstrakt: |
Autoimmune cytopenias are defined by autoantibodies' immune destruction of one or more blood elements. Most often it is autoimmune hemolytic anemia or immune thrombocytopenia or both that define Evans syndrome. It may be secondary to infection or to underlying pathology such as systemic autoimmune disease or primary immunodeficiency, especially when it becomes chronic over several years. Primary Immunodeficiencies or inborn errors of immunity (IEI) are no longer defined solely by infections: autoimmunity is part of the clinical features of several of these diseases. It is dominated by autoimmune cytopenias, in particular, immune thrombocytopenia (ITP) and autoimmune hemolytic anaemia (AIHA). The challenges for the clinician are the situations where autoimmune cytopenias are chronic, recurrent and/or refractory to the various long-term therapeutic options. Most of these therapies are similar in action and generally consist of non-mediated immune suppression or modulation. In these situations, primary Immunodeficiencies must be diagnosed as soon as possible to allow the initiation of a targeted treatment and to avoid several ineffective therapeutic lines. |
