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DISSEMINATED BACILLUS-CALMETTE-GUÉRIN INFECTIONS AND PRIMARY IMMUNODEFICIENCY DISORDERS IN SINGAPORE: A SINGLE CENTER

Rina Yue Ling Ong1, Su-Wan Bianca Chan2, Siu Jun Chew3

  • 1Department of Pharmacy, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229899, Singapore.

International Journal of Infectious Diseases : IJID : Official Publication of the International Society for Infectious Diseases
|June 5, 2020
PubMed

Insights

Disseminated Bacillus Calmette-Guérin (BCG) disease in children often indicates underlying primary immunodeficiency disorders (PIDs). Early immunology evaluation and hematopoietic stem cell transplant (HSCT) improve outcomes for BCGosis.

Area of Science:

  • Pediatric Immunology
  • Infectious Diseases
  • Genetics

Background:

  • Disseminated Bacillus Calmette-Guérin (BCG) disease, or BCGosis, is a significant complication in children with primary immunodeficiency disorders (PIDs).
  • Early identification and management are crucial for improving patient outcomes.

Purpose of the Study:

  • To investigate the clinical characteristics, underlying immunodeficiencies, and treatment outcomes of children with disseminated BCG disease.
  • To evaluate the efficacy of different treatment regimens and hematopoietic stem cell transplant (HSCT) in managing BCGosis.

Main Methods:

  • A 15-year retrospective review of patients diagnosed with BCGosis was conducted.
  • Patient data including demographics, underlying PIDs, microbiological findings, treatment protocols, and outcomes were analyzed.

Main Results:

  • Ten patients with BCGosis were identified, predominantly male, with a median age of 3.8 months at symptom onset.
  • Common PIDs included Severe Combined Immunodeficiency (SCID), Mendelian Susceptibility to Mycobacterial Diseases (MSMD), and others.
  • High susceptibility of Mycobacterium bovis subsp BCG to ethambutol, streptomycin, and kanamycin was observed; mortality was 50.0%.

Conclusions:

  • Disseminated BCG disease necessitates prompt immunology evaluation to diagnose underlying immune defects.
  • A three-drug regimen combined with early hematopoietic stem cell transplant (HSCT) appears adequate for treating BCGosis and improving survival rates.
Abstract

Keywords:
BCGBCGosisBacillus Calmette-GuérinDisseminatedImmunodeficiencyMycobacterium bovis subsp BCGVaccine

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