Immunological Memory and Affinity Maturation After Vaccination in Patients With Propionic Acidemia

Abstract

Earlier studies have recommended routine childhood immunization in patients with propionic acidemia (PA); however, the literature presents insufficient data on the response to vaccines, notably specific IgG concentrations and avidity maturation, after measles, mumps, rubella (MMR), and diphtheria/tetanus (DiphtTe) vaccinations in this population. In patients with PA, cellular and humoral changes of the immune system (e.g. a decreased CD4+ T cell count, with a reversal of CD4/CD8 T cell ratio, a deficient gamma-globulin fraction, and in one case a decreased lymphocyte blastogenesis) have been reported. Former reports also detected pancytopenias accompanying febrile infections in PA patients. In the current study, we analyzed vaccine-specific IgG concentrations and avidity maturation after MMR and DiphtTe vaccinations in 10 patients with PA. Compared to gender and age matched controls, all 10 had protective IgG concentrations for at least one tested antigen, and in 6 out of 10 patients high relative avidity indices for measles and rubella were detected. In summary, the present study revealed a sufficient immune response and outcome, indicating an acceptable humoral memory in patients with PA after booster vaccinations.

BNT162b2 COVID-19 vaccination elicited protective robust immune responses in pediatric patients with inborn errors of metabolism

“…( 11 ) In another study, anti-tetanus IgG and anti-diphtheria IgG levels were remarkably low in two patients with maple syrup urine disease (MSUD) despite an appropriate vaccination schedule ( 12 ). Most recently, an adequate humoral immune response was reported in ten patients with propionic acidemia (PA) who were vaccinated against measles, mumps, rubella (MMR), and diphtheria/tetanus (DiphtTe) ( 10 ). In our study, all IEM patients were found to be completely Anti-SARS-CoV-2 IgG seropositive after the second dose of the vaccination.…”

“…A primary immunodeficiency may be the cardinal feature of an IEM, or some degree of immune deficiency may contribute to the clinical spectrum as the disease progresses ( 7 – 9 ). However, the impact of immune system alterations on immunogenicity and vaccine efficacy in IEM is limited ( 8 , 10 – 12 ). The BNT162b2 mRNA COVID-19 vaccine (Pfizer-BioNTech ® ) is used worldwide and has been shown to be safe, immunogenic, and protective in children and adolescents during the pandemic ( 13 ).…”