Immunologic analysis of HIV-uninfected taiwanese children with BCG-induced disease

Wen I. Lee, Fang Chen Liang, Jing Long Huang, Tang Her Jaing, Chi Huei Wang, Tzou Yien Lin, Yhu Chering Huang, Wei Lun Huang, Ruwen Jou, Meng Ying Hsieh, Ju Hsin Chia, Tsu Lan Wu

Research output: Contribution to journalArticle

8 Citations (Scopus)

Abstract

Objectives: The aim of this study was to evaluate immunity in HIV-uninfected children with bacille Calmette-Guerin-induced disease (BCG-ID) over an 8-year period, with particular emphasis on underlying diseases. Methods: Patient afflicted with BCG-ID proven by clinical courses, dermatologic features, pathology, specific polymerase chain reaction, and/or spoligotyping were enrolled between 2000 and 2007. Lymphocyte proliferation, polymorphonuclear function, interleukin (IL)-12/23-interferons (IFN)-γ circuit, and Toll-like receptor 2-associated signaling were investigated. Results: Of the 271,618 total live births who received the BCG vaccine, eight patients (seven males) with BCG-ID were enrolled during an 8-year period and presented as three disseminated, two distant, and three regional BCG-ID. Their age at onset ranged from 1 to 28 months. All had a vaccine-injection scar except for one with lower CD3 and natural killer cells, compatible with severe combined immunodeficiency (SCID) identified by IL-2 receptor common gamma chain (IL2RG) mutation (Arg226Lys). The other SCID patient with de novo IL2RG mutation (Trp74Gly) had more recurrent infections. The third patient with primary autoimmune neutropenia had disseminated BCG-ID extending to abdominal wall. The fourth patient with chronic mucocutaneous candidiasis had regional BCG-ID and impaired lymphocyte proliferation to Candida and BCG antigens. No defective evidence of polymorphonuclear functions, IL-12/23-IFN-γ circuit, and Toll-like receptor 2-associated signaling was detected in the remaining four patients. Conclusion: Immunologic analysis in HIV-uninfected patients with BCG-ID reveals primary immunodeficiency diseases, especially in those with deficiencies in T-cell and neutrophil functions observed in our cohort, including primary autoimmune neutropenia and chronic mucocutaneous candidiasis.

Original languageEnglish
Pages (from-to)319-329
Number of pages11
JournalJournal of Clinical Immunology
Volume29
Issue number3
DOIs
Publication statusPublished - May 2009
Externally publishedYes

    Fingerprint

Keywords

  • Bacille Calmette-Guerin (BCG)
  • Chronic mucocutaneous candidiasis (CMC)
  • IL-2 receptor common gamma chain (IL2RG)
  • Primary autoimmune neutropenia (PAN)
  • Primary immunodeficiency diseases (PIDD)
  • Severe combined immunodeficiency (SCID)
  • Taiwan

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

Lee, W. I., Liang, F. C., Huang, J. L., Jaing, T. H., Wang, C. H., Lin, T. Y., Huang, Y. C., Huang, W. L., Jou, R., Hsieh, M. Y., Chia, J. H., & Wu, T. L. (2009). Immunologic analysis of HIV-uninfected taiwanese children with BCG-induced disease. Journal of Clinical Immunology, 29(3), 319-329. https://doi.org/10.1007/s10875-008-9265-5