Disseminated BCG in an infant with interleukin-12 receptor B1 (IL12RB1) deficiency

Abstract

Although neonatal vaccination with bacille Calmette–Guérin (BCG) is considered to be safe, complications with disseminated disease are associated with underlying immuno-deficiency disorders. A BCG-vaccinated 4-month-old girl of Sri Lankan parentage developed progressive left axillary lymphadenopathy and severe bronchopneumonia. Lymph node biopsy demonstrated epithelioid granulomata and acid-fast bacilli. An older sibling had had a similar clinical presentation and the outcome had been fatal. Investigation for immuno-deficiency detected complete IL12RB1 deficiency. Full recovery followed a prolonged course of anti-tuberculous chemotherapy. She was put on lifelong isoniazid prophylaxis. In HIV-negative infants with unusual complications related to BCG vaccination, a primary immuno-deficiency disorder should be considered.

Keywords:

• Neonatal BCG vaccination
• Mendelian susceptibility to mycobacterial disease
• Interleukin-12 receptor B1 (IL12RB1) deficiency

Acknowledgments

We thank Dr Shiromi Perera, consultant histopathologist, Lady Ridgeway Children’s Hospital Colombo and Dr Daham H. de Silva, paediatric registrar, for assistance in managing the patient. Dr Gabriela Barcenas-Morales was supported by a sabbatical grant from PASPA, Universidad Nacional Autonoma de Mexico.