Childhood isolated C3d-positive AIHA: favorable prognosis but rare relapse or associated immunodeficiency

Marion Blachez; Nathalie Aladjidi; Thomas Modot; Helder Fernandes; Nathalie Garnier; Pascal Chastagner; Sophie Bayart; Corinne Armari-Alla; Christophe Piguet; Marlène Pasquet; Catherine Paillard; Claire Pluchart; Corinne Guitton; Caroline Thomas; Vincent Barlogis; Isabelle Pellier; Jean-Louis Stephan; Joy Benadiba; Eric Jeziorski; Marianna Deparis; Liana Carausu; Nathalie Cheikh; Valérie Li-Thiao Te; Julien Lejeune; Aude Marie-Cardine; Frédéric Millot; Wadih Abou Chahla; Sophie Gay; Aurélia Alimi; Arnaud Petit; Martin Castelle; Despina Moshous; Capucine Picard; Frédéric Rieux-Laucat; Thierry Leblanc; Stéphane Ducassou; Guy Leverger; Mony Fahd; Sébastien Héritier

doi:10.1182/bloodadvances.2025017309

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Childhood isolated C3d-positive AIHA: favorable prognosis but rare relapse or associated immunodeficiency

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Childhood isolated C3d-positive AIHA: favorable prognosis but rare relapse or associated immunodeficiency

Marion Blachez, Nathalie Aladjidi, Thomas Modot, Helder Fernandes, Nathalie Garnier, Pascal Chastagner, Sophie Bayart, Corinne Armari-Alla, Christophe Piguet, Marlène Pasquet, …

Blood advances

21/10/2025

DOI: https://doi.org/10.1182/bloodadvances.2025017309

PMID: 41118608

Résumé

•Pediatric isolated C3d+ AIHA is mostly infection triggered and transient, and it rarely relapses.•IM may reveal primary immunodeficiencies such as autoimmune lymphoproliferative syndrome, warranting tailored evaluation at diagnosis. [Display omitted] Autoimmune hemolytic anemia (AIHA) with an isolated C3d+ direct antiglobulin test is a rare and understudied condition in children. It typically encompasses cold agglutinin syndrome and paroxysmal cold hemoglobinuria, both transient, infection-triggered disorders collectively referred to as cold AIHA. We report a national cohort of 142 pediatric patients with isolated C3d+ AIHA, representing 21.6% of all childhood AIHA cases enrolled in the French OBS’CEREVANCE cohort over a 32-year period. The median age at diagnosis was 3.2 years (male-to-female ratio, 1.3), and median follow-up was 2.8 years. Infectious symptoms were present in 63.4% of cases. At diagnosis, median hemoglobin was 6.4 g/dL; 69.7% of patients had inadequate reticulocytosis (bone marrow responsiveness index of <121), and 90.4% required transfusions. Eighteen patients (12.7%) had or developed immunopathological manifestations (IM) including 5 diagnosed with primary immunodeficiency (4 with autoimmune lymphoproliferative syndrome). Among 8 (5.6%) patients with relapsing disease, 6 had no IM at diagnosis but 4 developed IM at relapse. Nine patients were antinuclear antibodies (ANA) positive; none progressed to systemic lupus over a median follow-up of 4.9 years. Corticosteroids were administered to 82.4% of patients (median duration, 4.5 months), with no clear benefit over untreated patients regarding hospital stay or transfusion needs. No deaths were reported. In conclusion, pediatric isolated C3d+ AIHA generally follows a favorable course. However, a minority of patients may reveal underlying immune disorders, highlighting the importance of tailored evaluation at diagnosis. Cold agglutinin testing with thermal amplitude and Donath-Landsteiner testing, rarely performed in this cohort, warrant further study for their impact on diagnosis and clinical management.

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Titre: Childhood isolated C3d-positive AIHA: favorable prognosis but rare relapse or associated immunodeficiency
Créateurs - sans rôle: Marion Blachez - General Pediatrics and Pediatric Emergency Department, Saint Camille Hospital, Bry-sur-Marne, France
Nathalie Aladjidi - French reference center for pediatric autoimmune cytopenia (CEREVANCE) Pediatric Hemato-Immunology, CIC1401, INSERM CICP, Bordeaux University Hospital, Bordeaux, France
Thomas Modot - Etablissement Français du Sang, Saint Antoine Hospital, Paris, France
Helder Fernandes - French reference center for pediatric autoimmune cytopenia (CEREVANCE) Pediatric Hemato-Immunology, CIC1401, INSERM CICP, Bordeaux University Hospital, Bordeaux, France
Nathalie Garnier - Hospices Civils de Lyon
Pascal Chastagner - Centre Hospitalier Régional et Universitaire de Nancy
Sophie Bayart - Pediatric Hematology Unit, Rennes University Hospital, Rennes, France
Corinne Armari-Alla - Pediatric Hematology-Oncology Department, Grenoble University Hospital, Grenoble, France
Christophe Piguet - Pediatric Oncology Hematology Unit, Limoges University Hospital, Limoges, France
Marlène Pasquet - Pediatric Oncology Immunology Hematology Unit, Children's University Hospital, Toulouse, France
Catherine Paillard - Hôpitaux Universitaires de Strasbourg
Claire Pluchart - Pediatric Hematology-Oncology Unit, Centre Hospitalier Universitaire de Reims, Reims University Hospital, Reims, France
Corinne Guitton - Department of Pediatrics, Bicêtre University Hospital, Assistance publique–hôpitaux de Paris, Le Kremlin-Bicêtre, France
Caroline Thomas - Pediatric Hematology Unit, Nantes University Hospital, Nantes, France
Vincent Barlogis - Department of Pediatric Hematology, La Timone Hospital, Marseille University Hospital, Marseille, France
Isabelle Pellier - Pediatric Immuno-Hemato-Oncology, Angers University Hospital, CRCI2NA, UMR INSERM CNRS, Université d'Angers, Angers, France
Jean-Louis Stephan - Department of Pediatric Oncology, University Hospital of Saint Etienne, North Hospital, Saint Etienne, France
Joy Benadiba - Department of Hematology-Oncology Pediatrics, Nice University Hospital, Nice, France
Eric Jeziorski - Pediatric Oncology Hematology Unit, Arnaud de Villeneuve University Hospital, Montpellier, France
Marianna Deparis - Pediatric Oncology-Hematology Unit Department, Caen University Hospital, Caen, France
Liana Carausu - Department of Pediatric Hematology, Centre Hospitalier Universitaire de Brest, Brest, France
Nathalie Cheikh - Department of Pediatric Hematology-Oncology, Besançon University Hospital, Besançon, France
Valérie Li-Thiao Te - Department of Pediatric Hematology/Oncology, Amiens University Hospital, Amiens, France
Julien Lejeune - Department of Pediatric Hematology-Oncology, Clocheville Hospital, Tours University Hospital, Tours, France
Aude Marie-Cardine - Department of Pediatric Hematology and Oncology, Rouen University Hospital, Rouen, France
Frédéric Millot - Department of Pediatric Hematology, Poitiers University Hospital, Poitiers, France
Wadih Abou Chahla - Department of Pediatric Hematology, Jeanne de Flandre Hospital, Lille University Hospital, Lille, France
Sophie Gay - Sorbonne Université, INSERM, Centre de Recherche Saint-Antoine, UMR S938, CEREVANCE, Assistance Publique–Hôpitaux de Paris, Groupe Hospitalier Sorbonne Université, Hôpital Armand Trousseau, Paris, France
Aurélia Alimi - Sorbonne Université, INSERM, Centre de Recherche Saint-Antoine, UMR S938, CEREVANCE, Assistance Publique–Hôpitaux de Paris, Groupe Hospitalier Sorbonne Université, Hôpital Armand Trousseau, Paris, France
Arnaud Petit - Sorbonne Université, INSERM, Centre de Recherche Saint-Antoine, UMR S938, CEREVANCE, Assistance Publique–Hôpitaux de Paris, Groupe Hospitalier Sorbonne Université, Hôpital Armand Trousseau, Paris, France
Martin Castelle - Université Paris Cité
Despina Moshous - Pediatric Hematology-Immunology and Rheumatology Department, Université Paris Cité, Necker-Enfants Malades Hospital, Paris, France
Capucine Picard - Study Center for Primary Immunodeficiencies, Université Paris Cité, Necker-Enfants Malades University Hospital, Assistance Publique–Hôpitaux de Paris, Paris, France
Frédéric Rieux-Laucat - Laboratory of Immunogenetics of Pediatric Autoimmune Diseases, Institut Imagine, Université Paris Cité, INSERM, UMR S1163, Paris, France
Thierry Leblanc - French Reference Center for Pediatric Autoimmune Cytopenia (CEREVANCE), Pediatric Hematology Unit, Robert-Debré University Hospital, Assistance Publique–Hôpitaux de Paris, Paris, France
Stéphane Ducassou - French reference center for pediatric autoimmune cytopenia (CEREVANCE) Pediatric Hemato-Immunology, CIC1401, INSERM CICP, Bordeaux University Hospital, Bordeaux, France
Guy Leverger - Assistance Publique – Hôpitaux de Paris
Mony Fahd - French Reference Center for Pediatric Autoimmune Cytopenia (CEREVANCE), Pediatric Hematology Unit, Robert-Debré University Hospital, Assistance Publique–Hôpitaux de Paris, Paris, France
Sébastien Héritier - Sorbonne Université, INSERM, Centre de Recherche Saint-Antoine, UMR S938, CEREVANCE, Assistance Publique–Hôpitaux de Paris, Groupe Hospitalier Sorbonne Université, Hôpital Armand Trousseau, Paris, France
Détails de publication: Blood advances
Éditeur: Elsevier Inc
Identifiants: 99148012809311
Unité académique: Pathogenesis and Control of Chronic and Emerging Infections - PCCEI
Langue: English
Type de ressource: Journal article