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
Article

DOI: 10.1007/s10875-008-9265-5

Cite this article as:
Lee, WI., Liang, FC., Huang, JL. et al. J Clin Immunol (2009) 29: 319. doi:10.1007/s10875-008-9265-5

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.

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 

Abbreviations

BCG

bacille Calmette–Guerin

CMC

chronic mucocutaneous candidiasis

CGD

chronic granulomatous disease

IL2RG

IL-2 receptor common gamma chain

PBMCs

peripheral blood mononuclear cells

PIDD

primary immunodeficiency diseases

ROS

reactive oxygen species

SCID

severe combined immunodeficiency

Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  • Wen-I Lee
    • 1
    • 2
  • Fang-Chen Liang
    • 1
    • 2
  • Jing-Long Huang
    • 1
    • 2
  • Tang-Her Jaing
    • 1
    • 3
  • Chi-Huei Wang
    • 2
  • Tzou-Yien Lin
    • 4
  • Yhu-Chering Huang
    • 4
  • Wei-Lun Huang
    • 7
  • Ruwen Jou
    • 7
  • Meng-Ying Hsieh
    • 6
  • Ju-Hsin Chia
    • 5
  • Tsu-Lan Wu
    • 5
  1. 1.Primary Immunodeficiency Care and Research (PICAR) Institute, Chang Gung Memory Hospital, College of MedicineChang Gung UniversityTaoyuanTaiwan
  2. 2.Department of Pediatric Allergy, Immunology, and Rheumatology, Chang Gung Children’s HospitalChang Gung UniversityTaoyuanTaiwan
  3. 3.Pediatric Hematology and Oncology, Chang Gung Memory Hospital, College of MedicineChang Gung UniversityTaoyuanTaiwan
  4. 4.Pediatric Infection, Chang Gung Memory Hospital, College of MedicineChang Gung UniversityTaoyuanTaiwan
  5. 5.Clinical Pathology, Chang Gung Memory Hospital, College of MedicineChang Gung UniversityTaoyuanTaiwan
  6. 6.Graduate Institute of Medical Clinics, Chang Gung Memory Hospital, College of MedicineChang Gung UniversityTaoyuanTaiwan
  7. 7.Reference Laboratory of Mycobacteriology, Research and Diagnostic Center, Centers for Disease ControlDepartment of HealthTaipeiTaiwan

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