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Immunodeficiency Diseases01:25

Immunodeficiency Diseases

1.5K
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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Disorders of Hemostasis01:24

Disorders of Hemostasis

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Hemostasis, the process that stops bleeding after a blood vessel injury, is crucial for maintaining the integrity of the circulatory system. However, disorders of hemostasis can disrupt this delicate balance, leading to either excessive clotting or bleeding. These disorders can be broadly classified into thromboembolic disorders and bleeding disorders.
Thromboembolic Disorders
Two factors primarily cause thromboembolic conditions.
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Pulmonary Tuberculosis I01:29

Pulmonary Tuberculosis I

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Tuberculosis, often called TB, is a contagious illness primarily caused by Mycobacterium tuberculosis. It mainly affects the lung parenchyma but can also impact other body parts.
Causative Organism
The primary infectious agent causing tuberculosis is Mycobacterium tuberculosis, a slow-growing, acid-fast, aerobic rod that exhibits sensitivity to heat and ultraviolet light. Instances of Mycobacterium bovis and Mycobacterium avium contributing to the development of TB infection are rare.
Mode of...
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Humoral Immune Responses01:36

Humoral Immune Responses

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Overview
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Development of Immunocompetence01:22

Development of Immunocompetence

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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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Regulation of Hematopoietic Stem Cells01:01

Regulation of Hematopoietic Stem Cells

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All blood and immune cells are produced from the multipotent hematopoietic stem cells (HSCs) by the process of hematopoiesis. However, they all have a limited life span. In addition, many are depleted in immune surveillance or combatting an injury or infection. This makes blood one of the most regenerative tissues. Hematopoiesis helps replenish these blood and immune cells, restoring the body's normal functioning. However, overproduction of blood and immune cells can make them cancerous or...
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Updated: Nov 20, 2025

Simultaneous Quantification of T-Cell Receptor Excision Circles TRECs and K-Deleting Recombination Excision Circles KRECs by Real-time PCR
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Primary Immunodeficiency and Thrombocytopenia.

Maryam Mohtashami1,2, Azadehsadat Razavi2,3,4, Hassan Abolhassani2,5

  • 1Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran.

International Reviews of Immunology
|January 19, 2021
PubMed
Summary

Primary immunodeficiency (PI), or Inborn errors of immunity (IEI), can cause thrombocytopenia, a low platelet count. This review details IEI-related thrombocytopenia

Keywords:
Inborn errors of immunityinternational union of immunological societiesthrombocytopenia

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Area of Science:

  • Immunology
  • Hematology
  • Genetics

Background:

  • Primary immunodeficiency (PI), also known as Inborn Errors of Immunity (IEI), encompasses diverse disorders of immune system impairment.
  • Common manifestations include recurrent infections, autoimmunity, and malignancies.
  • Hematological abnormalities, particularly thrombocytopenia, can arise from various pathogenic mechanisms in IEI patients.

Purpose of the Study:

  • To review the manifestations, mechanisms, and therapeutic strategies for thrombocytopenia in patients with Inborn Errors of Immunity (IEI).
  • To consolidate information on thrombocytopenia as a complication across different classes of IEI, based on the International Union of Immunological Societies (IUIS) classification.

Main Methods:

  • Literature review focusing on primary and secondary thrombocytopenia in the context of IEI.
  • Analysis of existing data and classification systems (IUIS) to categorize IEI disorders associated with thrombocytopenia.

Main Results:

  • Thrombocytopenia is a recognized complication in various innate and adaptive immunodeficiency disorders within the IEI spectrum.
  • Pathogenic mechanisms leading to thrombocytopenia in IEI are diverse and depend on the specific underlying immunodeficiency.
  • This review provides a comprehensive overview of these manifestations, mechanisms, and treatment options.

Conclusions:

  • Thrombocytopenia is a significant hematological complication in Inborn Errors of Immunity (IEI).
  • Understanding the specific IEI class is crucial for elucidating the pathogenic mechanisms and guiding therapeutic interventions for thrombocytopenia.
  • Further research is warranted to optimize management strategies for IEI-associated thrombocytopenia.