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Chronic hypogammaglobulinemia after allogeneic stem cell transplantation and their treatment with subcutaneous immunoglobulin in pediatric patients.
Serra Font, Sara; López-Granados, Lucía; Sisinni, Luisa; Serna Berna, José Vicente; Martínez Martínez, Laura; Fernández de Gamarra-Martínez, Edurne; de la Calle Martín, Oscar; Badell Serra, Isabel.
Afiliação
  • Serra Font S; Unidad Pediátrica De Hematología, Oncología y Trasplante Hematopoyético, Hospital De La Santa Creu i Sant Pau, Barcelona, Spain. Electronic address: sserraf@santpau.cat.
  • López-Granados L; Unidad Pediátrica De Hematología, Oncología y Trasplante Hematopoyético, Hospital De La Santa Creu i Sant Pau, Barcelona, Spain.
  • Sisinni L; Unidad Pediátrica De Hematología, Oncología y Trasplante Hematopoyético, Hospital De La Santa Creu i Sant Pau, Barcelona, Spain.
  • Serna Berna JV; Unidad Pediátrica De Hematología, Oncología y Trasplante Hematopoyético, Hospital De La Santa Creu i Sant Pau, Barcelona, Spain; Instituto De Investigaciones Biomédicas Sant Pau (IIB Sant Pau), Universidad Autónoma De Barcelona, Barcelona, Spain.
  • Martínez Martínez L; Instituto De Investigaciones Biomédicas Sant Pau (IIB Sant Pau), Universidad Autónoma De Barcelona, Barcelona, Spain; Servicio De Inmunología, Hospital De La Santa Creu i Sant Pau, Barcelona, Spain.
  • Fernández de Gamarra-Martínez E; Servicio De Farmacia, Hospital De La Santa Creu i Sant Pau, Barcelona, Spain.
  • de la Calle Martín O; Instituto De Investigaciones Biomédicas Sant Pau (IIB Sant Pau), Universidad Autónoma De Barcelona, Barcelona, Spain; Servicio De Inmunología, Hospital De La Santa Creu i Sant Pau, Barcelona, Spain.
  • Badell Serra I; Unidad Pediátrica De Hematología, Oncología y Trasplante Hematopoyético, Hospital De La Santa Creu i Sant Pau, Barcelona, Spain; Instituto De Investigaciones Biomédicas Sant Pau (IIB Sant Pau), Universidad Autónoma De Barcelona, Barcelona, Spain.
An Pediatr (Engl Ed) ; 97(2): 103-111, 2022 Aug.
Article em En | MEDLINE | ID: mdl-35869014
INTRODUCTION: Hypogammaglobulinemia in the first months after allogeneic hematopoietic stem cell transplantation (HSCT) is common in paediatric patients. During this phase, replacement therapy with human immunoglobulin must be administered parenterally to prevent infections. In some cases, this hypogammaglobulinemia persists over time, which forces further treatment when the patient is usually no longer a carrier of a central line, making them ideal candidates for subcutaneous replacement therapy. There is little published literature describing the use of this method in paediatric patients undergoing HSCT, widely described in replacement treatment in children with primary immunodeficiencies with very good results. PATIENTS AND METHODS: An observational, descriptive, longitudinal and retrospective study is carried out. During the years 2008-2019, we evaluated all paediatric patients undergoing HSCT in our center with persistent chronic hypogammaglobulinemia (for over a year). The treatment phase with intravenous immunoglobulin (Privigen®) and the first four years of treatment with subcutaneous immunoglobulin (Hizentra®) are evaluated using a questionnaire. RESULTS: During the years 2008-2019, 175 patients underwent HSCT, 143 (82%) of whom exceeded three months after transplantation. Three (2%) of them had persistent hypogammaglobulinemia. All three share factors described in the literature involved in immune reconstitution. After analysing the questionnaire, it is observed that switching from intravenous to subcutaneous gammaglobulin has involved a great improvement in their quality of life. CONCLUSIONS: The origin of chronic hypogammaglobulinemia in our patients shows different factors and cannot be attributed to a single cause. Due to the limited number of patients no conclusions can be drawn at the population level. We have been able to observe that replacement treatment with Hizentra 20% has been as effective as the intravenous administration without evidence of an increase in bacterial infections. Furthermore, it has also led to an improvement in quality of life and increased comfort, as the patients themselves have stated.
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Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Transplante de Células-Tronco Hematopoéticas / Agamaglobulinemia / Doença Enxerto-Hospedeiro Tipo de estudo: Etiology_studies / Observational_studies / Risk_factors_studies Limite: Child / Humans Idioma: En Revista: An Pediatr (Engl Ed) Ano de publicação: 2022 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Transplante de Células-Tronco Hematopoéticas / Agamaglobulinemia / Doença Enxerto-Hospedeiro Tipo de estudo: Etiology_studies / Observational_studies / Risk_factors_studies Limite: Child / Humans Idioma: En Revista: An Pediatr (Engl Ed) Ano de publicação: 2022 Tipo de documento: Article