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Related Concept Videos

Disorders of Leukocytes01:27

Disorders of Leukocytes

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Leukocyte disorders can lead to either leukopenia, characterized by an abnormally low leukocyte count, or leukocytosis, marked by a very high leukocyte number.
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B Cell Activation and Differentiation01:24

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The adaptive immune response, a sophisticated defense mechanism, relies on the activation and differentiation of B lymphocytes, or B cells. These processes enable our bodies to mount a tailored response against specific pathogens such as bacteria, free virus particles, toxins, and parasites.
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Cells of the Adaptive Immune Response01:23

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The T and B lymphocytes of the adaptive immune system develop from common lymphoid progenitor cells in the bone marrow. These progenitors give rise to precursors that eventually develop into both T and B lymphocytes. As these precursors mature, they gain the ability to detect and respond to foreign antigens in the body, a process known as immunocompetence. Additionally, these precursors acquire self-tolerance, a process that ensures they do not react to self-antigens. This intricate system...
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Disorders of erythrocytes, or red blood cells (RBCs), include a range of conditions affecting their number, shape, or function.
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Immunodeficiency disorders are conditions in which the immune system's ability to fight infectious disease and cancer is compromised or entirely absent. The immune system comprises a complex network of cells, tissues, and organs that work together to protect the body from potentially harmful invaders. When this system is deficient or not functioning properly, it leaves the body susceptible to infections, diseases, or other complications.
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Primary Lymphoid Organs01:16

Primary Lymphoid Organs

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Primary lymphoid organs are pivotal in the formation, development, and maturation of lymphocytes, the white blood cells that serve as the backbone of our immune system. This crucial function underscores their fundamental role in maintaining our overall health and immunity. The two primary lymphoid organs of prime importance are the red bone marrow and the thymus.
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Isolation of Precursor B-cell Subsets from Umbilical Cord Blood
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B Cell Disorders in Children-Part I.

Bailee Gilchrist1, William K Dolen2

  • 1Department of Pediatrics, Allergy-Immunology and Pediatric Rheumatology Division, Medical College of Georgia at Augusta University, 1120 15th Street, Augusta, GA, 30912, USA.

Current Allergy and Asthma Reports
|June 25, 2020
PubMed
Summary
This summary is machine-generated.

Enhanced genetic testing reveals gene defects in pediatric antibody deficiencies like agammaglobulinemia and common variable immunodeficiency (CVID). This advances diagnosis and understanding of B cell disorders.

Keywords:
AgammaglobulinemiaAntibody deficiencyB cell disordersCommon variable immunodeficiencyImmunodeficiency in children

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Area of Science:

  • Pediatric Immunology
  • Clinical Genetics
  • Molecular Biology

Background:

  • Antibody deficiencies, including agammaglobulinemia and common variable immunodeficiency (CVID), are significant pediatric health concerns.
  • Historically, these conditions were broadly classified, often dichotomously.

Purpose of the Study:

  • To review the gene defects underlying antibody deficiencies in children.
  • To describe the clinical manifestations associated with these genetic defects.
  • To highlight the impact of advanced genetic testing on diagnosing B cell disorders.

Main Methods:

  • Review of current literature on genetic testing and immunodeficiencies.
  • Analysis of newly discovered monogenetic defects.
  • Correlation of genetic findings with clinical presentations.

Main Results:

  • Enhanced genetic testing has identified specific gene defects in both agammaglobulinemia and CVID.
  • A strict dichotomous classification of B cell disorders is increasingly inadequate due to novel monogenetic defect discoveries.
  • Advances in genetic technology facilitate more precise diagnoses and improved genetic counseling.

Conclusions:

  • The classification of B cell disorders requires re-evaluation in light of genetic discoveries.
  • Genetic testing is crucial for accurate diagnosis and understanding of pediatric antibody deficiencies.
  • Further research is expected to uncover additional gene defects and improve our comprehension of B cell development.