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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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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.
The red bone marrow is a soft, spongy tissue nestled in the interior of long bones such as the humerus and femur. It is the site...
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Secondary Lymphoid Organs01:15

Secondary Lymphoid Organs

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Secondary organs, including lymph nodes, the spleen, and mucosa-associated lymphoid tissue (MALT), work harmoniously to protect us from disease and infection.
The spleen is a vital organ in the lymphatic system, nestled in the upper left side of the abdomen. It is composed of two primary regions: the red pulp and the white pulp, each having distinct functions. The red pulp performs a significant role in blood filtration. It efficiently purges the blood of old or damaged red blood cells and...
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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.
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...
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Lymphoid Cells and Tissues01:18

Lymphoid Cells and Tissues

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Lymphoid cells and tissues are integral to the immune system, which is crucial in maintaining our body's defense against harmful pathogens. They form the building blocks of lymphoid organs, which include the spleen, thymus, and lymph nodes.
Lymphoid cells consist of various types of immune system cells. These include B and T lymphocytes, which are responsible for producing antibodies and killing infected cells, respectively. Dendritic cells act as messengers between the innate and adaptive...
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Cells of the Adaptive Immune Response01:23

Cells of the Adaptive Immune Response

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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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Separation of Immune Cell Subpopulations in Peripheral Blood Samples from Children with Infectious Mononucleosis
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Lymphocyte Subset Abnormalities in Pediatric-Onset Common Variable Immunodeficiency.

Ismail Ogulur1,2, Ayca Kiykim1, Dilek Baser1

  • 1Division of Pediatric Allergy-Immunology, Faculty of Medicine, Marmara University, Istanbul, Turkey.

International Archives of Allergy and Immunology
|January 6, 2020
PubMed
Summary
This summary is machine-generated.

Pediatric-onset Common Variable Immunodeficiency (CVID) shows distinct lymphocyte subset abnormalities, including decreased B and T cells, linked to organ damage like bronchiectasis and chronic diarrhea. These findings aid in understanding CVID prognosis.

Keywords:
Common variable immunodeficiencyEnd organ involvementLymphocyte subsetsPediatric patients

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Area of Science:

  • Immunology
  • Pediatric Medicine
  • Clinical Research

Background:

  • Common Variable Immunodeficiency (CVID) is a primary immunodeficiency characterized by hypogammaglobulinemia and recurrent infections.
  • B-cell abnormalities in CVID can predict organ involvement and prognosis.
  • Pediatric-onset CVID is rare, and its lymphocyte subset abnormalities require further investigation.

Purpose of the Study:

  • To identify lymphocyte subset abnormalities in pediatric-onset CVID.
  • To associate these abnormalities with specific end-organ involvement in pediatric CVID patients.

Main Methods:

  • Assessed absolute numbers and percentages of B, T, and NK cells in pediatric CVID patients (10-16 years) and compared them to age-matched healthy controls.
  • Compared pediatric CVID patients with adult CVID patients (>16 years).
  • Evaluated associations between lymphocyte subsets and organ involvement.

Main Results:

  • Pediatric CVID patients exhibited decreased total and memory B cells, CD4+ T cells, naive T cells (CD4+CD45RA+), and recent thymic emigrant (RTE) cells, with increased CD8+CD45RO+ memory T cells.
  • Adult CVID patients uniquely showed high frequencies of activated and double-negative T cells.
  • Reduced B and NK cells, along with increased CD8+ T cells, correlated with bronchiectasis.
  • Low serum IgA and decreased naive T/RTE cells were risk factors for chronic diarrhea in pediatric CVID.

Conclusions:

  • Specific B- and T-lymphocyte abnormalities are identified in pediatric-onset CVID.
  • These cellular abnormalities are associated with end-organ manifestations in pediatric CVID patients.