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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

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.
There are three main causes of immunodeficiency disorders...
Development of Immunocompetence01:22

Development of Immunocompetence

The initiation of cell-mediated immunity can be observed as early as the third month of fetal growth, with active antibody-mediated immunity following approximately one month later.
The initial cells that migrate from the fetal thymus settle within the skin and epithelial tissues lining the mouth, digestive tract, and in females, the uterus and vagina. These cells, including skin-based dendritic cells, serve as antigen-presenting cells, playing a key role in T cell activation.
Subsequent T...
Special Features of Adaptive Immunity01:20

Special Features of Adaptive Immunity

The adaptive immune system, a crucial component of the overall immune response, offers a highly specialized defense against pathogens. It involves specific cell types and features, enabling it to combat infections effectively and efficiently.
The primary cell types involved in adaptive immunity are T cells and B cells. Each type has a unique role in defending the body against pathogens. T cells are responsible for cell-mediated immunity. They identify and eliminate infected cells directly,...
Disorders of Leukocytes01:27

Disorders of Leukocytes

Leukocyte disorders can lead to either leukopenia, characterized by an abnormally low leukocyte count, or leukocytosis, marked by a very high leukocyte number.
Leukopenia may result from bone marrow disorders, autoimmune diseases, and infectious diseases. For example, conditions such as multiple myeloma and aplastic anemia can impair the bone marrow's ability to produce adequate leukocytes. Similarly, autoimmune diseases like lupus and viral infections such as HIV can prompt the immune system...
Autoimmune Disorders01:29

Autoimmune Disorders

Autoimmune diseases are a group of disorders in which the body's immune system mistakenly attacks its own cells, tissues, and organs. This results from an overactive immune response against substances and tissues normally present in the body. Let's delve into the concept and mechanism of autoimmune diseases from an immune system point of view, explore different causes and examples of such diseases, and discuss potential solutions.
Concept and Mechanism of Autoimmune Diseases
The immune system...
Hypersensitivity Reactions: Immune-Complex Reactions01:19

Hypersensitivity Reactions: Immune-Complex Reactions

Type III hypersensitivity reactions occur when antigen–antibody complexes form and activate the complement system. Normally, these complexes help the clearance of antigens by phagocytes and red blood cells. However, when large numbers of immune complexes are present, they can deposit in tissues—particularly in the walls of blood vessels—leading to inflammation and tissue injury. These deposits trigger complement activation and neutrophil recruitment, resulting in serum sickness, a systemic...

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Whole Blood Assay with Dual Co-Stimulation for Antigen-Specific Analysis of Host Immunity to Fungal and Viral Pathogens
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Whole Blood Assay with Dual Co-Stimulation for Antigen-Specific Analysis of Host Immunity to Fungal and Viral Pathogens

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Common variable immunodeficiency: a multifaceted and puzzling disorder.

Astrid Bergbreiter1, Ulrich Salzer

  • 1Department of Rheumatology and Clinical Immunology, Medical School, University Hospital Freiburg, Hugstetterstrasse 55, 79106 Freiburg, Germany. astrid.bergbreiter@uniklinik-freiburg.de

Expert Review of Clinical Immunology
|May 19, 2010
PubMed
Summary
This summary is machine-generated.

Common variable immunodeficiencies (CVIDs) are complex immune disorders. Research highlights B cell defects in most patients, suggesting subtypes for better understanding and treatment.

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Area of Science:

  • Immunology
  • Genetics
  • Clinical Medicine

Background:

  • Common variable immunodeficiencies (CVIDs) are heterogeneous disorders marked by hypogammaglobulinemia and recurrent infections.
  • While specific genetic defects (e.g., ICOS, TACI, CD19) are identified, they explain only a fraction of CVID cases.
  • Numerous immunological abnormalities in T cells, B cells, and antigen-presenting cells are observed in CVID patients.

Purpose of the Study:

  • To explore the immunological and clinical heterogeneity of CVIDs.
  • To understand the relationship between genetic defects, immune cell dysfunction, and clinical manifestations.
  • To identify distinct subtypes of CVID for improved classification and management.

Main Methods:

  • Analysis of immunological phenotypes and functional abnormalities in T cells, B cells, and antigen-presenting cells.
  • Review of genetic data identifying causative or risk-increasing factors.
  • Examination of clinical phenotypes, markers, and long-term outcomes from multicenter studies.

Main Results:

  • Impaired terminal differentiation of peripheral B cells affects approximately 80% of CVID patients, offering a basis for classification.
  • Multicenter studies have identified clinical phenotypes and predictive immunological markers.
  • Long-term outcome data from large CVID patient cohorts are being reported.

Conclusions:

  • Identifying distinct immunological and clinical subtypes is crucial for understanding CVID pathogenesis.
  • Elucidating the interplay between genetic defects, immune abnormalities, and clinical phenotypes will advance CVID research.
  • This knowledge is essential for improving diagnostic and therapeutic strategies for CVID patients.