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Outcome of BCG Vaccination in ADA-SCID Patients: A 12-Patient Series

Daniele Canarutto,Chiara Oltolini,Federica Barzaghi,Valeria Calbi,Maddalena Migliavacca,Francesca Tucci,Vera Gallo,Giulia Consiglieri,Francesca Ferrua,Salvatore Recupero,Maria Celia Cervi,Hamoud Al-Mousa,Anna Pituch-Noworolska,Chiara Tassan Din,Paolo Scarpellini,Paolo Silvani,Claudia Fossati,Miriam Casiraghi,Daniela Maria Cirillo,Antonella Castagna,Maria Ester Bernardo,Alessandro Aiuti,Maria Pia Cicalese

BIOMEDICINES（2023）

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Abstract
Vaccination with Bacillus Calmette-Guerin (BCG) can be harmful to patients with combined primary immunodeficiencies. We report the outcome of BCG vaccination in a series of twelve patients affected by adenosine deaminase deficiency (ADA-SCID). BCG vaccination resulted in a very high incidence of complications due to uncontrolled replication of the mycobacterium. All patients who developed BCG-related disease were treated successfully and remained free from recurrence of disease. We recommend the prompt initiation of enzyme replacement therapy and secondary prophylaxis to reduce the risk of BCG-related complications in ADA-SCID patients.
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Key words
enzyme replacement therapy, isoniazid, rifampin, tuberculosis, newborn screening, gene therapy
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