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Related Concept Videos

Immunodeficiency Diseases01:25

Immunodeficiency Diseases

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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.
There are three main causes of immunodeficiency...
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Special Features of Adaptive Immunity01:20

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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.
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Cytomegalovirus (CMV) disease is caused by human cytomegalovirus, a double-stranded DNA virus of the Herpesviridae family. While primary CMV infection is often asymptomatic in immunocompetent individuals, the virus can cause severe disease in neonates and immunocompromised patients. CMV is the most common cause of congenital viral infection in the United States, and a major pathogen in solid organ and hematopoietic stem cell transplant recipients.CMV is transmitted via bodily fluids, sexual...
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Development of Immunocompetence01:22

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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...
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Antigens Involved in Adaptive Immunity01:26

Antigens Involved in Adaptive Immunity

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An antigen is any substance the immune system identifies as foreign and potentially harmful to the body, prompting an immune response. Antigens have two functional properties: immunogenicity and reactivity. Immunogenicity is the ability of an antigen to stimulate a specific immune response. At the same time, reactivity describes the antigen's ability to react with the cells and antibodies produced in response to it.
Complete Antigens
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Hypersensitivity Reactions: Immune-Complex Reactions01:19

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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...
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Development of an IFN-γ ELISpot Assay to Assess Varicella-Zoster Virus-specific Cell-mediated Immunity Following Umbilical Cord Blood Transplantation
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[Combined variable immunodeficiency with unusal features. A case report].

F De Charry1, A Peterschmitt Tonetti2, C De Charry3

  • 1Service de médecine interne-oncologie, HIA Desgenettes, 108, boulevard Pinel, 69003 Lyon, France.

La Revue De Medecine Interne
|March 18, 2014
PubMed
Summary
This summary is machine-generated.

This case report details the first known association between combined variable immunodeficiency (CVID) and large granular lymphocyte (LGL) leukemia. The patient developed GLILD before being diagnosed with LGL leukemia.

Keywords:
Atteinte pulmonaireCombined variable immunodeficiencyDéficit immunitaire commun variableGranulomatoseGranulomatosisLarge granular lymphocyte (LGL) leukemiaLeucémie à grands lymphocytes à grainsLung disease

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Area of Science:

  • Immunology
  • Hematology
  • Pulmonology

Background:

  • Combined variable immunodeficiency (CVID) is a recognized clinical condition.
  • Granulomatous disease is a known complication of CVID.
  • The co-occurrence of CVID with large granular lymphocyte (LGL) leukemia has not been previously documented.

Observation:

  • A 50-year-old female patient with CVID and granulomatous disease was monitored.
  • The patient progressed to develop granulomatous lymphocytic interstitial lung disease (GLILD).
  • Subsequently, the patient was diagnosed with LGL leukemia.

Findings:

  • This report presents the first documented instance of a patient with CVID also developing LGL leukemia.
  • The clinical course involved the progression from CVID and granulomatous disease to GLILD, and finally to LGL leukemia.

Implications:

  • This case highlights a potential, previously unrecognized association between CVID and LGL leukemia.
  • Further research may be warranted to explore the pathobiology and clinical significance of this comorbidity.
  • Clinicians should consider LGL leukemia in the differential diagnosis of CVID patients presenting with specific hematologic or pulmonary manifestations.