Abstract |
The presence of active viral infections has an impact on the prognosis of patients undergoing hematopoietic stem cell transplantation (HSCT). Nevertheless, the number of reports of cytomegalovirus infection in patients with inborn errors of immunity (IEI) who undergo HSCT is relatively low. To analyze the effect of cytomegalovirus infection acquired prior to curative treatment on patient survival in 123 children with IEI. An observational and retrospective study was performed with patients younger than 18 years diagnosed with IEI who were candidates for HSCT, gene therapy, or thymus transplantation at five hospitals in Spain between 2008 and 2019. We included 123 children, 25 infected by cytomegalovirus prior to undergoing curative treatment (20.3%). At IEI diagnosis, 24 of the patients were already infected, 21 of whom had symptomatic cytomegalovirus disease (87%), while the other three patients developed disease before undergoing curative treatment. The patients with cytomegalovirus infection had higher mortality than those without (p = 0.006). Fourteen patients developed refractory cytomegalovirus infection (56%), all of whom died, while no patients with non-refractory infection died (p = 0.001) All deaths that occurred before curative treatment and three of the five after the treatment were attributed to cytomegalovirus. Patients with refractory cytomegalovirus disease had the highest pre-HSCT mortality rate (64.3%), compared with the non-infected children and those with non-refractory cytomegalovirus disease (10.1%) (p < 0.0001). CONCLUSION: Prevention and prompt control of cytomegalovirus infection, together with early HSCT/gene therapy, are crucial for improving the prognosis in children with IEI. WHAT IS KNOWN: WHAT IS NEW: • In children with inborn errors of immunity and indication of HSCT, refractory cytomegalovirus disease is associated with a very high mortality rate, compared with non-infected children and those with non-refractory cytomegalovirus disease. • In patients with novel transplantation indications, the presence and treatment response of CMV infection should be considered to decide the best possible moment for HSCT.
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Authors | Teresa Del Rosal, Cristian Quintana-Ortega, Angela Deyá-Martinez, Pere Soler-Palacín, Walter Alfredo Goycochea-Valdivia, Nerea Salmón, Antonio Pérez-Martínez, Laia Alsina, Andrea Martín-Nalda, Laura Alonso, Olaf Neth, Luz Yadira Bravo-Gallego, Luis Ignacio Gonzalez-Granado, Ana Mendez-Echevarria |
Journal | European journal of pediatrics
(Eur J Pediatr)
Vol. 181
Issue 11
Pg. 3889-3898
(Nov 2022)
ISSN: 1432-1076 [Electronic] Germany |
PMID | 36102997
(Publication Type: Journal Article, Observational Study)
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Copyright | © 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature. |
Topics |
- Child
- Cytomegalovirus
(genetics)
- Cytomegalovirus Infections
- Hematopoietic Stem Cell Transplantation
(adverse effects)
- Humans
- Retrospective Studies
- Transplantation, Homologous
(adverse effects)
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