Recurrent Bronchiolitis

  • Sevgi Köstel Bal
  • Figen Doğu


  • The most common infectious agents in combined immunodeficiencies are intracellular pathogens, such as cytomegalovirus, herpes simplex virus, Pneumocystis jirovecii, Salmonella and Cryptosporidium.

  • Immunophenotyping in patients with MHC class II deficiency shows absent or very low HLA-DR expression on B lymphocytes, monocytes or activated T lymphocytes, with reduced CD4+ cell counts leading to an inverted CD4+/CD8+ ratio


MHC class II deficiency HLA-DR CD4+/CD8+ ratio Pneumocystis jirovecii 


  1. 1.
    Ochs HD, Smith CIE, Puck J. Primary immunodeficiency diseases: a molecular and genetic approach. 3rd ed. Oxford/New York: Oxford University Press; 2014.Google Scholar
  2. 2.
    Heimall J, Buckley RH, Puck J, Fleisher TA, Gennery AR, Haddad E, Neven B, Slatter M, Roderick S, Baker KS, Dietz AC, Duncan C, Griffith LM, Notarangelo L, Pulsipher MA, Cowan MJ. Recommendations for screening and management of late effects in patients with severe combined immunodeficiency after allogenic hematopoietic cell transplantation: a consensus statement from the second pediatric blood and marrow transplant consortium international conference on late effects after pediatric HCT. Biol Blood Marrow Transplant. 2017;23(8):1229–40.CrossRefGoogle Scholar
  3. 3.
    Gennery A. Recent advances in understanding and treating chronic granulomatous disease. F1000Research. 2017;6:1427.CrossRefGoogle Scholar
  4. 4.
    Farrokhi S, Shabani M, Aryan Z, Zoghi S, Krolo A, Boztug K, Rezaei N. MHC class II deficiency: report of a novel mutation and special review. Allergol Immunopathol. 2018;46(3):263–75.CrossRefGoogle Scholar
  5. 5.
    Hanna S, Etzioni A. MHC class I and II deficiencies. J Allergy Clin Immunol. 2014;134(2):269–75.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Sevgi Köstel Bal
    • 1
  • Figen Doğu
    • 1
  1. 1.Ankara University School of Medicine, Department of Pediatric Allergy and ImmunologyAnkaraTurkey

Personalised recommendations