Autoimmune cytopenias in children: When to think of primary immunodeficiency?

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Published Feb 11, 2024
https://doi.org/10.62438/tunismed.v102i1.4503
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Vol. 102 No. 1 (2024): Tunis med jan 2024

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Mohamed Hbibi
Department of Haematology-Oncology Paediatric SHOP , Hassan II University Hospital, Fez. Morocco
Mounira El Alaoui El Hanafi
Department of Haematology-Oncology Paediatric SHOP , Ibn Rochd University Hospital, Casablanca. Morocco
Zakaria Kasmi
Laboratory of Clinical Immunology, Inflammation and Allergy (LICIA), Faculty of Medicine and Pharmacy Casablanca. Morocco
Hind Ouair
Laboratory of Clinical Immunology, Inflammation and Allergy (LICIA), Faculty of Medicine and Pharmacy Casablanca. Morocco
Sarra Benmiloud
Department of Haematology-Oncology Paediatric SHOP , Hassan II University Hospital, Fez. Morocco
Fatima Ailal
Department of Infectious diseases and clinical immunology paediatric, Ibn Rochd hospital Casablanca. Morocco
Moustapha Hida
Department of Paediatrics , Hassan II hospital Fez. Morocco
Ahmed Aziz Bousfiha
Department of infectious diseases and clinical immunology paediatric , Ibn Rochd hospital Casablanca. Morocco

Abstract

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.

Keywords:

IEI , Autoimmunity, Thrombocytopenia, Purpura, Leukopenia, Human, Evans Syndrome

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