Frontiers in Pediatrics (Oct 2023)
Mycophenolate mofetil for autoimmune cytopenias in children: high rates of response in inborn errors of immunity
- Rubén Berrueco,
- Rubén Berrueco,
- Rubén Berrueco,
- Elisa González-Forster,
- Elisa González-Forster,
- Angela Deya-Martinez,
- Angela Deya-Martinez,
- Angela Deya-Martinez,
- María Solsona,
- María Solsona,
- Ana García-García,
- Ana García-García,
- Ana García-García,
- Joan Calzada-Hernández,
- Luo Yiyi,
- Luo Yiyi,
- Alexandru Vlagea,
- Alexandru Vlagea,
- Anna Ruiz-Llobet,
- Anna Ruiz-Llobet,
- Anna Ruiz-Llobet,
- Laia Alsina,
- Laia Alsina,
- Laia Alsina,
- Laia Alsina
Affiliations
- Rubén Berrueco
- Pediatric Hematology Department, Hospital Sant Joan de Déu, Barcelona, Spain
- Rubén Berrueco
- Institut de Recerca Sant Joan de Déu de Barcelona (IRSJD), Barcelona, Spain
- Rubén Berrueco
- Instituto Nacional de Investigación Biomédica en Enfermedades Raras (CIBER ER), Instituto de Salud Carlos III, Madrid, Spain
- Elisa González-Forster
- Pediatric Hematology Department, Hospital Sant Joan de Déu, Barcelona, Spain
- Elisa González-Forster
- Institut de Recerca Sant Joan de Déu de Barcelona (IRSJD), Barcelona, Spain
- Angela Deya-Martinez
- Institut de Recerca Sant Joan de Déu de Barcelona (IRSJD), Barcelona, Spain
- Angela Deya-Martinez
- Clinical Immunology and Primary Immunodeficiencies Unit, Pediatric Allergy and Clinical Immunology Department, Hospital Sant Joan de Déu, Barcelona, Spain
- Angela Deya-Martinez
- Clinical Immunology Unit, Hospital Sant Joan de Déu-Hospital Clínic Barcelona, Barcelona, Spain
- María Solsona
- Pediatric Hematology Department, Hospital Sant Joan de Déu, Barcelona, Spain
- María Solsona
- Institut de Recerca Sant Joan de Déu de Barcelona (IRSJD), Barcelona, Spain
- Ana García-García
- Institut de Recerca Sant Joan de Déu de Barcelona (IRSJD), Barcelona, Spain
- Ana García-García
- Clinical Immunology and Primary Immunodeficiencies Unit, Pediatric Allergy and Clinical Immunology Department, Hospital Sant Joan de Déu, Barcelona, Spain
- Ana García-García
- Clinical Immunology Unit, Hospital Sant Joan de Déu-Hospital Clínic Barcelona, Barcelona, Spain
- Joan Calzada-Hernández
- Pediatric Rheumatology Unit, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain
- Luo Yiyi
- Clinical Immunology and Primary Immunodeficiencies Unit, Pediatric Allergy and Clinical Immunology Department, Hospital Sant Joan de Déu, Barcelona, Spain
- Luo Yiyi
- Clinical Immunology Unit, Hospital Sant Joan de Déu-Hospital Clínic Barcelona, Barcelona, Spain
- Alexandru Vlagea
- Clinical Immunology Unit, Hospital Sant Joan de Déu-Hospital Clínic Barcelona, Barcelona, Spain
- Alexandru Vlagea
- Immunology Department, Centre of Biomedical Diagnosis, Hospital Clínic, Barcelona, Spain
- Anna Ruiz-Llobet
- Pediatric Hematology Department, Hospital Sant Joan de Déu, Barcelona, Spain
- Anna Ruiz-Llobet
- Institut de Recerca Sant Joan de Déu de Barcelona (IRSJD), Barcelona, Spain
- Anna Ruiz-Llobet
- Instituto Nacional de Investigación Biomédica en Enfermedades Raras (CIBER ER), Instituto de Salud Carlos III, Madrid, Spain
- Laia Alsina
- Institut de Recerca Sant Joan de Déu de Barcelona (IRSJD), Barcelona, Spain
- Laia Alsina
- Clinical Immunology and Primary Immunodeficiencies Unit, Pediatric Allergy and Clinical Immunology Department, Hospital Sant Joan de Déu, Barcelona, Spain
- Laia Alsina
- Clinical Immunology Unit, Hospital Sant Joan de Déu-Hospital Clínic Barcelona, Barcelona, Spain
- Laia Alsina
- Department of Surgery and Surgical Specializations, Facultat de Medicina I Ciències de la Salut, Universitat de Barcelona, Barcelona, Spain
- DOI
- https://doi.org/10.3389/fped.2023.1174671
- Journal volume & issue
-
Vol. 11
Abstract
Second-line treatments of autoimmune cytopenias (AC) are not well-defined in children. Mycophenolate mofetil (MMF) is an immunosuppressant agent that has been demonstrated to be safe and effective in this setting. A retrospective observational study was conducted in 18 children with prolonged AC who received MMF, in order to describe clinical and biological markers of response. The overall response rate of MMF at 20–30 mg/kg per day was 73.3%. All patients with Evans syndrome (n = 9) achieved complete response. Among the patients with monolineage AC (n = 9), those with an underlying inborn errors of immunity (IEI), tended to respond better to MMF. No biological markers related to treatment response were found. Rather, lymphocyte subpopulations proved useful for patient selection as a marker suggestive of IEI along with immunoglobulin-level determination.
Keywords
- pediatrics
- autoimmune hemolytic anemia
- autoimmune thrombocytopenia
- Evans syndrome
- autoimmune diseases
- mycophenolate mofetil