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 Esteves; Nermeen 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

doi:10.1016/j.jaci.2014.02.028

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^*

^*Corresponding author for this work

Child Health

Research output: Contribution to journal › Article › peer-review

119 Citations (Scopus)

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

Original language	English
Pages (from-to)	1134-1141
Number of pages	8
Journal	Journal of Allergy and Clinical Immunology
Volume	133
Issue number	4
DOIs	https://doi.org/10.1016/j.jaci.2014.02.028
Publication status	Published - 2014

Keywords

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

ASJC Scopus subject areas

Immunology and Allergy
Immunology

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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

BCG vaccination in patients with severe combined immunodeficiency : Complications, risks, and vaccination policies. / Marciano, Beatriz E.; Huang, Chiung Yu; Joshi, Gyan; Rezaei, Nima; Carvalho, Beatriz Costa; Allwood, Zoe; Ikinciogullari, Aydan; Reda, Shereen M.; Gennery, Andrew; Thon, Vojtech; Espinosa-Rosales, Francisco; Al-Herz, Waleed; Porras, Oscar; Shcherbina, Anna; Szaflarska, Anna; Kiliç, Şebnem; Franco, Jose L.; Gómez Raccio, Andrea C.; Roxo, Persio; Esteves, Isabel; Galal, Nermeen; Grumach, Anete Sevciovic; Al-Tamemi, Salem; Yildiran, Alisan; Orellana, Julio C.; Yamada, Masafumi; Morio, Tomohiro; Liberatore, Diana; Ohtsuka, Yoshitoshi; Lau, Yu Lung; Nishikomori, Ryuta; Torres-Lozano, Carlos; Mazzucchelli, Juliana T L; Vilela, Maria M S; Tavares, Fabiola S.; 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; Bezrodnik, Liliana; Marques, Jose Gonçalo; Pereira, María I.; Listello, Viviana; Slatter, Mary A.; Nademi, Zohreh; Kowalczyk, Danuta; Fleisher, Thomas A.; Davies, Graham; Neven, Bénédicte; Rosenzweig, Sergio D.

In: Journal of Allergy and Clinical Immunology, Vol. 133, No. 4, 2014, p. 1134-1141.

Research output: Contribution to journal › Article › peer-review

Marciano, BE, Huang, CY, Joshi, G, Rezaei, N, Carvalho, BC, Allwood, Z, Ikinciogullari, A, Reda, SM, Gennery, A, Thon, V, Espinosa-Rosales, F, Al-Herz, W, Porras, O, Shcherbina, A, Szaflarska, A, Kiliç, Ş, Franco, JL, Gómez Raccio, AC, Roxo, P, Esteves, I, Galal, N, Grumach, AS, Al-Tamemi, S, Yildiran, A, Orellana, JC, Yamada, M, Morio, T, Liberatore, D, Ohtsuka, Y, Lau, YL, Nishikomori, R, Torres-Lozano, C, Mazzucchelli, JTL, Vilela, MMS, Tavares, FS, Cunha, L, Pinto, JA, Espinosa-Padilla, SE, Hernandez-Nieto, L, Elfeky, RA, Ariga, T, Toshio, H, Dogu, F, Cipe, F, Formankova, R, Nuñez-Nuñez, ME, Bezrodnik, L, Marques, JG, Pereira, MI, Listello, V, Slatter, MA, Nademi, Z, Kowalczyk, D, Fleisher, TA, Davies, G, Neven, B & Rosenzweig, SD 2014, 'BCG vaccination in patients with severe combined immunodeficiency: Complications, risks, and vaccination policies', Journal of Allergy and Clinical Immunology, vol. 133, no. 4, pp. 1134-1141. https://doi.org/10.1016/j.jaci.2014.02.028

Marciano, Beatriz E. ; Huang, Chiung Yu ; Joshi, Gyan ; Rezaei, Nima ; Carvalho, Beatriz Costa ; Allwood, Zoe ; Ikinciogullari, Aydan ; Reda, Shereen M. ; Gennery, Andrew ; Thon, Vojtech ; Espinosa-Rosales, Francisco ; Al-Herz, Waleed ; Porras, Oscar ; Shcherbina, Anna ; Szaflarska, Anna ; Kiliç, Şebnem ; Franco, Jose L. ; Gómez Raccio, Andrea C. ; Roxo, Persio ; Esteves, Isabel ; Galal, Nermeen ; Grumach, Anete Sevciovic ; Al-Tamemi, Salem ; Yildiran, Alisan ; Orellana, Julio C. ; Yamada, Masafumi ; Morio, Tomohiro ; Liberatore, Diana ; Ohtsuka, Yoshitoshi ; Lau, Yu Lung ; Nishikomori, Ryuta ; Torres-Lozano, Carlos ; Mazzucchelli, Juliana T L ; Vilela, Maria M S ; Tavares, Fabiola S. ; 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 ; Bezrodnik, Liliana ; Marques, Jose Gonçalo ; Pereira, María I. ; Listello, Viviana ; Slatter, Mary A. ; Nademi, Zohreh ; Kowalczyk, Danuta ; Fleisher, Thomas A. ; Davies, Graham ; Neven, Bénédicte ; Rosenzweig, Sergio D. / BCG vaccination in patients with severe combined immunodeficiency : Complications, risks, and vaccination policies. In: Journal of Allergy and Clinical Immunology. 2014 ; Vol. 133, No. 4. pp. 1134-1141.

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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 ",

keywords = "BCG, hematopoietic stem cell transplant, immune reconstitution syndrome, mycobacteria, newborn screening, Primary immunodeficiency, severe combined immunodeficiency, vaccine",

author = "Marciano, {Beatriz E.} and Huang, {Chiung Yu} and Gyan Joshi and Nima Rezaei and Carvalho, {Beatriz Costa} and Zoe Allwood and Aydan Ikinciogullari and Reda, {Shereen M.} and Andrew Gennery and Vojtech Thon and Francisco Espinosa-Rosales and Waleed Al-Herz and Oscar Porras and Anna Shcherbina and Anna Szaflarska and {\c S}ebnem Kili{\c c} and Franco, {Jose L.} and {G{\'o}mez Raccio}, {Andrea C.} and Persio Roxo and Isabel Esteves and Nermeen Galal and Grumach, {Anete Sevciovic} and Salem Al-Tamemi and Alisan Yildiran and Orellana, {Julio C.} and Masafumi Yamada and Tomohiro Morio and Diana Liberatore and Yoshitoshi Ohtsuka and Lau, {Yu Lung} and Ryuta Nishikomori and Carlos Torres-Lozano and Mazzucchelli, {Juliana T L} and Vilela, {Maria M S} and Tavares, {Fabiola S.} and Luciana Cunha and Pinto, {Jorge A.} and Espinosa-Padilla, {Sara E.} and Leticia Hernandez-Nieto and Elfeky, {Reem A.} and Tadashi Ariga and Heike Toshio and Figen Dogu and Funda Cipe and Renata Formankova and Nu{\~n}ez-Nu{\~n}ez, {M. Enriqueta} and Liliana Bezrodnik and Marques, {Jose Gon{\c c}alo} and Pereira, {Mar{\'i}a I.} and Viviana Listello and Slatter, {Mary A.} and Zohreh Nademi and Danuta Kowalczyk and Fleisher, {Thomas A.} and Graham Davies and B{\'e}n{\'e}dicte Neven and Rosenzweig, {Sergio D.}",

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T1 - BCG vaccination in patients with severe combined immunodeficiency

T2 - Complications, risks, and vaccination policies

AU - Marciano, Beatriz E.

AU - Huang, Chiung Yu

AU - Joshi, Gyan

AU - Rezaei, Nima

AU - Carvalho, Beatriz Costa

AU - Allwood, Zoe

AU - Ikinciogullari, Aydan

AU - Reda, Shereen M.

AU - Gennery, Andrew

AU - Thon, Vojtech

AU - Espinosa-Rosales, Francisco

AU - Al-Herz, Waleed

AU - Porras, Oscar

AU - Shcherbina, Anna

AU - Szaflarska, Anna

AU - Kiliç, Şebnem

AU - Franco, Jose L.

AU - Gómez Raccio, Andrea C.

AU - Roxo, Persio

AU - Esteves, Isabel

AU - Galal, Nermeen

AU - Grumach, Anete Sevciovic

AU - Al-Tamemi, Salem

AU - Yildiran, Alisan

AU - Orellana, Julio C.

AU - Yamada, Masafumi

AU - Morio, Tomohiro

AU - Liberatore, Diana

AU - Ohtsuka, Yoshitoshi

AU - Lau, Yu Lung

AU - Nishikomori, Ryuta

AU - Torres-Lozano, Carlos

AU - Mazzucchelli, Juliana T L

AU - Vilela, Maria M S

AU - Tavares, Fabiola S.

AU - Cunha, Luciana

AU - Pinto, Jorge A.

AU - Espinosa-Padilla, Sara E.

AU - Hernandez-Nieto, Leticia

AU - Elfeky, Reem A.

AU - Ariga, Tadashi

AU - Toshio, Heike

AU - Dogu, Figen

AU - Cipe, Funda

AU - Formankova, Renata

AU - Nuñez-Nuñez, M. Enriqueta

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AU - Marques, Jose Gonçalo

AU - Pereira, María I.

AU - Listello, Viviana

AU - Slatter, Mary A.

AU - Nademi, Zohreh

AU - Kowalczyk, Danuta

AU - Fleisher, Thomas A.

AU - Davies, Graham

AU - Neven, Bénédicte

AU - Rosenzweig, Sergio D.

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N2 - 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

AB - 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

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KW - hematopoietic stem cell transplant

KW - immune reconstitution syndrome

KW - mycobacteria

KW - newborn screening

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KW - severe combined immunodeficiency

KW - vaccine

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

Abstract

Keywords

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