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Related Concept Videos

Humoral Immune Responses01:36

Humoral Immune Responses

Overview
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...
Hypersensitivities01:30

Hypersensitivities

Hypersensitivity, also known as a hypersensitivity reaction or allergic reaction, is a condition where the body's immune system reacts abnormally to a foreign substance. Such substances, that cause hypersensitivity are referred to as an allergen, could be something typically harmless to most people, like pollen or certain foods.
Types of Hypersensitivities
Hypersensitivity reactions are categorized into four types: Type 1, Type 2, Type 3, and Type 4. Each type has a distinct mechanism...
Hypersensitivity Reactions: Cytolytic Reactions01:01

Hypersensitivity Reactions: Cytolytic Reactions

Type II hypersensitivity involves IgG and IgM antibodies targeting cell surface antigens, leading to cell destruction. This can occur through complement activation, antibody-dependent cell-mediated cytotoxicity (ADCC), or acting as opsonins for phagocytosis. When excessive, these reactions cause significant tissue damage.Drug-induced hemolytic anemia is a common example, where drugs like penicillin or cephalosporins bind to red blood cells, forming drug-protein complexes. These complexes...
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...
Hypersensitivity Reactions: Delayed Hypersensitivity Reactions01:29

Hypersensitivity Reactions: Delayed Hypersensitivity Reactions

Delayed-Type Hypersensitivity (DTH), or Type IV hypersensitivity, is a cell-mediated immune response. It occurs when T cells, rather than antibodies, mediate a reaction to specific antigens. It is characterized by a delayed onset (1-2 days) and involves the recruitment of macrophages to the inflammation site.The initiation of a DTH response begins with the sensitization of T cells. During this phase, which lasts at least 1-2 weeks, antigen-specific T cells are activated, clonally expanded, and...

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Related Experiment Video

Updated: Jun 15, 2026

Analysis of Somatic Hypermutation in the JH4 intron of Germinal Center B cells from Mouse Peyer's Patches
09:35

Analysis of Somatic Hypermutation in the JH4 intron of Germinal Center B cells from Mouse Peyer's Patches

Published on: April 20, 2021

Update on the hyper immunoglobulin M syndromes.

E Graham Davies1, Adrian J Thrasher

  • 1Centre for Immunodeficiency, Institute of Child Health, London, UK. davieg1@gosh.nhs.uk

British Journal of Haematology
|February 26, 2010
PubMed
Summary

Hyper-immunoglobulin M syndromes (HIGM) involve defects in immunoglobulin class switch recombination (CSR). Treatment varies, with some patients benefiting from immunoglobulin replacement therapy, while others may need bone marrow transplantation.

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Analysis of Somatic Hypermutation in the JH4 intron of Germinal Center B cells from Mouse Peyer's Patches
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Area of Science:

  • Immunology
  • Genetics
  • Molecular Biology

Background:

  • Hyper-immunoglobulin M syndromes (HIGM) are a diverse group of genetic disorders.
  • These conditions are characterized by defects in immunoglobulin class switch recombination (CSR), potentially affecting somatic hypermutation (SHM).

Purpose of the Study:

  • To classify Hyper-immunoglobulin M syndromes based on underlying molecular defects.
  • To outline the clinical manifestations and treatment strategies for HIGM.
  • To discuss potential complications and future therapeutic directions.

Main Methods:

  • Classification of HIGM based on CD40 signaling defects versus intrinsic B cell defects in CSR.
  • Review of current therapeutic approaches including immunoglobulin replacement and bone marrow transplantation.
  • Discussion of gene therapy as a future prospect.

Main Results:

  • HIGM can result from CD40 signaling defects, leading to humoral immunodeficiency and opportunistic infections, or intrinsic B cell defects causing pure humoral immunodeficiency.
  • HIGM can also present as part of broader DNA repair defects or antibody deficiencies.
  • CD40 signaling defects may necessitate bone marrow transplantation, while defective CSR mechanisms often respond well to immunoglobulin replacement therapy.

Conclusions:

  • Hyper-immunoglobulin M syndromes are heterogeneous genetic disorders impacting humoral immunity.
  • Treatment strategies are tailored to the specific defect, ranging from immunoglobulin replacement to bone marrow transplantation.
  • Complications include autoimmunity, lymphoid hyperplasia, and an increased risk of lymphoid malignancy.