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

Author:

Canarutto Daniele123ORCID,Oltolini Chiara4,Barzaghi Federica23,Calbi Valeria23,Migliavacca Maddalena23,Tucci Francesca23ORCID,Gallo Vera23,Consiglieri Giulia23,Ferrua Francesca23,Recupero Salvatore23,Cervi Maria Celia5,Al-Mousa Hamoud6,Pituch-Noworolska Anna7,Tassan Din Chiara4,Scarpellini Paolo4,Silvani Paolo8,Fossati Claudia2,Casiraghi Miriam2,Cirillo Daniela Maria9ORCID,Castagna Antonella14,Bernardo Maria Ester123,Aiuti Alessandro123,Cicalese Maria Pia123

Affiliation:

1. Faculty of Medicine and Surgery, Vita-Salute S. Raffaele University, 20132 Milan, Italy

2. San Raffaele Telethon Institute for Gene Therapy (SR-Tiget), IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy

3. Pediatric Immunohematology Unit and BMT Program, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy

4. Clinic of Infectious Diseases, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy

5. Pediatric Infectious Diseases Division, Department of Pediatrics, Ribeirao Preto Medical School, University of Sao Paulo, Sao Paulo 05508-000, Brazil

6. Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh 11564, Saudi Arabia

7. Department of Immunology, University Children’s Hospital, 30-663 Krakow, Poland

8. Department of Anesthesia and Critical Care, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy

9. Emerging Bacterial Pathogens Unit, Division of Immunology, Transplantation and Infectious Diseases, IRCCS San Raffaele Scientific Institute, 20132 Milan, Italy

Abstract

Vaccination with Bacillus Calmette–Guérin (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.

Funder

Fondazione Telethon

Publisher

MDPI AG

Subject

General Biochemistry, Genetics and Molecular Biology,Medicine (miscellaneous)

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