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

Beatriz E. Marciano, Chiung Yu Huang, Gyan Joshi, Nima Rezaei, Beatriz Costa Carvalho, Zoe Allwood, Aydan Ikinciogullari, Shereen M. Reda, Andrew Gennery, Vojtech Thon, Francisco Espinosa-Rosales, Waleed Al-Herz, Oscar Porras, Anna Shcherbina, Anna Szaflarska, Şebnem Kiliç, Jose L. Franco, Andrea C. Gómez Raccio, Persio Roxo, Isabel EstevesNermeen Galal, Anete Sevciovic Grumach, Salem Al-Tamemi, Alisan Yildiran, Julio C. Orellana, Masafumi Yamada, Tomohiro Morio, Diana Liberatore, Yoshitoshi Ohtsuka, Yu Lung Lau, Ryuta Nishikomori, Carlos Torres-Lozano, Juliana T.L. Mazzucchelli, Maria M.S. Vilela, Fabiola S. Tavares, Luciana Cunha, Jorge A. Pinto, Sara E. Espinosa-Padilla, Leticia Hernandez-Nieto, Reem A. Elfeky, Tadashi Ariga, Heike Toshio, Figen Dogu, Funda Cipe, Renata Formankova, M. Enriqueta Nuñez-Nuñez, Liliana Bezrodnik, Jose Gonçalo Marques, María I. Pereira, Viviana Listello, Mary A. Slatter, Zohreh Nademi, Danuta Kowalczyk, Thomas A. Fleisher, Graham Davies, Bénédicte Neven, Sergio D. Rosenzweig

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1134-1141
Number of pages8
JournalJournal of Allergy and Clinical Immunology
Volume133
Issue number4
DOIs
StatePublished - Apr 2014

Keywords

  • BCG
  • Primary immunodeficiency
  • hematopoietic stem cell transplant
  • immune reconstitution syndrome
  • mycobacteria
  • newborn screening
  • severe combined immunodeficiency
  • vaccine

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

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  • Cite this

    Marciano, B. E., Huang, C. Y., Joshi, G., Rezaei, N., Carvalho, B. C., Allwood, Z., Ikinciogullari, A., Reda, S. M., Gennery, A., Thon, V., Espinosa-Rosales, F., Al-Herz, W., Porras, O., Shcherbina, A., Szaflarska, A., Kiliç, Ş., Franco, J. L., Gómez Raccio, A. C., Roxo, P., ... Rosenzweig, S. D. (2014). BCG vaccination in patients with severe combined immunodeficiency: Complications, risks, and vaccination policies. Journal of Allergy and Clinical Immunology, 133(4), 1134-1141. https://doi.org/10.1016/j.jaci.2014.02.028