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Pulmonary Manifestations of Combined T- and B-Cell Immunodeficiencies

  • Andrew R. GenneryEmail author
Chapter

Abstract

The immune system has evolved in coexistence with microbes over billions of years with the purpose of providing efficient but self-limited host defense against the biotic and abiotic environment, during which self-tolerance is maintained. The three key components of human immune defense are (i) barriers, (ii) innate immune response, and (iii) adaptive immune response. The key elements of the adaptive response are T-lymphocytes and B-lymphocytes, and defects in T-lymphocytes alone or both T- and B-lymphocytes give rise to combined immunodeficiency. Because the lung is a major interface between the internal and external environment, so pulmonary infection is a frequent consequence of combined immunodeficiency, but other pulmonary manifestations are also recognized. This chapter will concentrate on clinical patterns of presentation, as well as describe specific diseases in detail. Specific respiratory syndromes associated with specific diseases will be highlighted.

Keywords

Primary immunodeficiency diseases Severe combined immunodeficiency Severe combined immunodeficiency due to adenosine deaminase deficiency Combined immunodeficiency Reticular dysgenesis X-linked combined immunodeficiency diseases Pulmonary disease 

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Institute of Cellular Medicine, Newcastle UniversityNewcastle upon TyneUK
  2. 2.Paediatric Immunology and Haematopoietic Stem Cell Transplantation Unit, Great North Childrens’ HospitalNewcastle upon TyneUK

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