OBS'CEREVANCE: French Cohort of Pediatric Autoimmune Cytopenia

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Mit akar kideríteni a vizsgálat

From 2004, OBS'CEREVANCE is a national real-world prospective clinical cohort of patients with auto-immune cytopenia of pediatric-onset : Immune thrombocytopenia (ITP), Autoimmune Hemolytic anemia (AIHA), or Evans syndrome (all bi or tri cytopenias). Thanks to the collaboration of the 30 French pediatric hematologic centers, this cohort supports all of the Rare Disease Centre CEREVANCE (Centre de Référence National des Cytopénies Auto-Immunes de l'Enfant) missions for care, education and research. Specifically, this original unbiased database allows to describe the long-term health of adult patients, to identify the heterogenous genetic underlying pathophysiologic contexts, and to study the benefit-risk balance of treatments, including the growing development of targeted therapies.

A vizsgálat 3 csoportja

• Immune thrombocytopenia (ITP)

  Immune thrombocytopenia (ITP) : defined according to the international working group criteria (Rodeghiero et al., Blood 2009).

  Kezelés: data collection

• Autoimmune Hemolytic anemia (AIHA)

  Autoimmune haemolytic anaemia (AIHA) : Hb \< 110 g/L with a positive direct antiglobulin test (DAT) and at least one of the following haemolysis criteria: reticulocyte count \> 120 G/L, free bilirubin \> 17 mmol/L, or haptoglobin \< 10 mg/dL.

  Kezelés: data collection

• Evans syndrome (all bi or tri cytopenias)

  Evans syndrome (ES) : simultaneous (less than 1 month) or sequential association of at least two autoimmune cytopenia among ITP, AIHA and autoimmune neutropenia (AIN).

  Kezelés: data collection