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White Blood Cell and Immunodeficiency Disorders

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Molecular Pathology of Hematolymphoid Diseases

Part of the book series: Molecular Pathology Library ((MPLB,volume 4))

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Abstract

The human immune system comprises cellular and humoral elements that sense danger, and respond to threat in both nonspecific and antigen-specific ways that eliminate the offending agents and restore homeostasis. The innate immune system refers to those elements that recognize danger and activate the immune response. The cellular components of innate immunity include monocytes, dendritic cells, macrophages, mast cells, neutrophils, natural killer (NK) cells, and eosinophils. The humoral components include the complement proteins, C-reactive protein, and mannose lectin binding protein. In addition to activation of the immune system via cytokines and elaboration of effector molecules (such as interferons), some of the cells of the innate system process foreign antigen and present it to the adapative immune system. The adaptive immune system comprises the T and B cell compartments. Cytotoxic T cells kill infected cells; helper T cells provide support for the production of antibody by B cells; and regulatory T cells moderate the adaptive responses and prevent the emergence of autoimmunity. Primary immunodeficiencies derive from mutations in the genes involved in this elaborate host response. These mutations may occur at any phase of the immune response (i.e., from danger recognition to synthesis of high affinity antibody). The molecular consequences of these mutations lead to undue susceptibility to infection, autoimmunity, and, in some instances, malignancy.

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Authors and Affiliations

  1. Department of Pediatrics, University of California, San Diego, La Jolla, CA, USA

    John F. Bastian MD

  2. Department of Pediatrics, University of North Carolina, Chapel Hill, NC, USA

    Michelle Hernandez MD

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  1. Dept. Pathology & Laboratory Medicine, University of North Carolina, Chapel Hill, 27599-7525, USA

    Cherie H. Dunphy

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Bastian, J.F., Hernandez, M. (2010). White Blood Cell and Immunodeficiency Disorders. In: Dunphy, C. (eds) Molecular Pathology of Hematolymphoid Diseases. Molecular Pathology Library, vol 4. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-5698-9_38

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