BCG vaccination in patients with severe combined immunodeficiency: Complications, risks, and vaccination policies

Authors
Gómez Raccio, Andrea C.; Orellana, Julio Cesar; Liberatore, Diana; Bezrodnik, Liliana; Marciano, Beatriz E.; Huang, Chiung Yu; Joshi, Gyan; Rezaei, Nima; Costa Carvalho, Beatriz; Cunha, Luciana; Pinto, Jorge A.; Espinosa Padilla, Sara E.; Hernandez Nieto, Leticia; Elfeky, Reem A.; Ariga, Tadashi; Toshio, Heike; Dogu, Figen; Cipe, Funda; Formankova, Renata; Nuñez Nuñez, M. Enriqueta; Gonçalo Marques, Jose; Pereira, María I.; Listello, Viviana; Slatter, Mary A.; Nademi, Zohreh; Kowalczyk, Danuta; Fleisher, Thomas A.; Davies, Graham; Neven, Bénédicte; Rosenzweig, Sergio D.
Publication Year
2014
Language
English
Format
article
Status
Published version
Description
BACKGROUND: Severe combined immunodeficiency (SCID) is a syndrome characterized by profound T-cell deficiency. BCG vaccine is contraindicated in patients with SCID. Because most countries encourage BCG vaccination at birth, a high percentage of patients with SCID are vaccinated before their immune defect is detected. OBJECTIVES: We sought to describe the complications and risks associated with BCG vaccination in patients with SCID. METHODS: An extensive standardized questionnaire evaluating complications, therapeutics, and outcomes regarding BCG vaccination in patients given a diagnosis of SCID was widely distributed. Summary statistics and association analysis was performed. RESULTS: Data on 349 BCG-vaccinated patients with SCID from 28 centers in 17 countries were analyzed. Fifty-one percent of the patients had BCG-associated complications, 34% disseminated and 17% localized (a 33,000- and 400-fold increase, respectively, over the general population). Patients receiving early vaccination (≤1 month) showed an increased prevalence of complications (P = .006) and death caused by BCG-associated complications (P < .0001). The odds of experiencing complications among patients with T-cell numbers of 250/μL or less at diagnosis was 2.1 times higher (95% CI, 1.4-3.4 times higher; P = .001) than among those with T-cell numbers of greater than 250/μL. BCG-associated complications were reported in 2 of 78 patients who received antimycobacterial therapy while asymptomatic, and no deaths caused by BCG-associated complications occurred in this group. In contrast, 46 BCG-associated deaths were reported among 160 patients treated with antimycobacterial therapy for a symptomatic BCG infection (P < .0001). CONCLUSIONS: BCG vaccine has a very high rate of complications in patients with SCID, which increase morbidity and mortality rates. Until safer and more efficient antituberculosis vaccines become available, delay in BCG vaccination should be considered to protect highly vulnerable populations from preventable complications.
Fil: Gómez Raccio, Andrea C.. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños ; Argentina
Fil: Orellana, Julio Cesar. Provincia de Córdoba. Hospital de Niños de la Santísima Trinidad. División de Alergia e Inmunología Clínica; Argentina
Fil: Liberatore, Diana. Hospital Italiano; Argentina
Fil: Bezrodnik, Liliana. Gobierno de la Ciudad de Buenos Aires. Hospital General de Niños ; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina
Fil: Marciano, Beatriz E.. National Institutes of Health; Estados Unidos
Fil: Huang, Chiung Yu. National Institutes of Health; Estados Unidos
Fil: Joshi, Gyan. National Institutes of Health; Estados Unidos
Fil: Rezaei, Nima. Teheran University of Medical Sciences. Children's Medical Center Hospital. Pediatric Center of Excellence; Irán
Fil: Costa Carvalho, Beatriz. Federal University of São Paulo; Brasil
Fil: Cunha, Luciana. Federal University of Minas Gerais; Brasil
Fil: Pinto, Jorge A.. Federal University of Minas Gerais; Brasil
Fil: Espinosa Padilla, Sara E.. Secretaría de Salud. Instituto Nacional de Pediatría; México
Fil: Hernandez Nieto, Leticia. Secretaría de Salud. Instituto Nacional de Pediatría; México
Fil: Elfeky, Reem A.. Ain Shams University; Egipto
Fil: Ariga, Tadashi. Hokkaido University Graduate School of Medicine; Japón
Fil: Toshio, Heike. Kyoto University Hospital; Japón
Fil: Dogu, Figen. Ankara University Medical School; Turquía
Fil: Cipe, Funda. Ankara University Medical School; Turquía
Fil: Formankova, Renata. Charles University; República Checa. University Hospital Motol; República Checa
Fil: Nuñez Nuñez, M. Enriqueta. Western National Medical Center; México
Fil: Gonçalo Marques, Jose. Santa María Hospital. Lisbon Academic Center; Portugal
Fil: Pereira, María I.. Provincia de Córdoba. Hospital de Niños de la Santísima Trinidad. División de Alergia e Inmunología Clínica; Argentina
Fil: Listello, Viviana. Provincia de Córdoba. Hospital de Niños de la Santísima Trinidad. División de Alergia e Inmunología Clínica; Argentina
Fil: Slatter, Mary A.. Great North Children's Hospital; Reino Unido
Fil: Nademi, Zohreh. Great North Children's Hospital; Reino Unido
Fil: Kowalczyk, Danuta. Children's University Hospital. Department of Clinical Immunology and Transplantology; Polonia
Fil: Fleisher, Thomas A.. National Institutes of Health; Estados Unidos
Fil: Davies, Graham. Great Ormond Street Hospital for Children; Reino Unido
Fil: Neven, Bénédicte. Necker Hospital. Immunology-Hematology and Rheumatology Service; Francia
Fil: Rosenzweig, Sergio D.. National Institute of Health. National Institute of Allergy and Infectious. Laboratory of Host Defenses. Primary Immunodeficiency Clinic and Infectious Diseases Susceptibility Unit; Estados Unidos
Subject
Hematopoietic stem cell transplant
IL-2 receptor g
Recombination-activating gene
Severe combined immunodeficiency
Primary immunodeficiency
Vaccine
BCG
Immune reconstitution syndrome
Mycobacteria
Newborn screening
Medicina Critica y de Emergencia
Medicina Clínica
CIENCIAS MÉDICAS Y DE LA SALUD
Access level
Open access
License
https://creativecommons.org/licenses/by-nc-nd/2.5/ar/
Repository
CONICET Digital (CONICET)
Institution
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identifier
oai:ri.conicet.gov.ar:11336/34105