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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...
B Cell Activation and Differentiation01:24

B Cell Activation and Differentiation

The adaptive immune response, a sophisticated defense mechanism, relies on the activation and differentiation of B lymphocytes, or B cells. These processes enable our bodies to mount a tailored response against specific pathogens such as bacteria, free virus particles, toxins, and parasites.
When naive B cells encounter a specific antigen that can bind to the B cell receptor (BCR) on their surface, they undergo sensitization to respond to the antigen's presence. Sensitization begins with...
Transcytosis of IgG01:15

Transcytosis of IgG

Transcytosis is the process in which molecules are internalized by endocytosis, transported across the cell, and released through exocytosis from the opposite end of the cell. Molecules such as insulin, immunoglobulins, and certain nutrients are transferred through the recycling endosomes by recycling and transcytosis.
IgG molecules from a mother undergo transcytosis starting around 13 weeks of gestation. The amount of IgG transferred and entering the fetal blood circulation increases with...

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Immunoglobulin Gene Sequence Analysis In Chronic Lymphocytic Leukemia: From Patient Material To Sequence Interpretation
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Immunoglobulin Gene Sequence Analysis In Chronic Lymphocytic Leukemia: From Patient Material To Sequence Interpretation

Published on: November 26, 2018

Revisiting X-linked agammaglobulinemia.

Hirokazu Kanegane1, Kay Tanita2, Madoka Nishimura2,3

  • 1Department of Child Health and Development, Graduate School of Medical and Dental Sciences, Institute of Science Tokyo, Tokyo, Japan.

Journal of Human Immunity
|May 22, 2026
PubMed
Summary
This summary is machine-generated.

X-linked agammaglobulinemia (XLA) is a common inborn error of immunity. This review highlights unresolved issues in XLA, including genetic variants, infections, and potential future treatments like stem cell transplantation.

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Area of Science:

  • Immunology
  • Genetics
  • Infectious Diseases

Background:

  • X-linked agammaglobulinemia (XLA) is a well-established inborn error of immunity (IEI) with an incidence of 1:100,000–1:200,000.
  • Characterized by recurrent bacterial infections, hypo/agammaglobulinemia, and B-cell deficiency, its pathogenesis is understood, but several aspects remain unclear.

Purpose of the Study:

  • To review and discuss unresolved issues in XLA.
  • To highlight areas for future research and potential therapeutic advancements.

Main Methods:

  • Literature review of existing studies on XLA.
  • Discussion of current understanding and emerging challenges in XLA management.

Main Results:

  • Identified unresolved issues include noncoding BTK variants, contiguous deletion syndrome, Helicobacter infection, neurodegeneration, renal involvement, and malignancies.
  • Current primary treatment, immunoglobulin replacement therapy, has not changed.
  • Allogeneic hematopoietic cell transplantation shows promise but requires further investigation.

Conclusions:

  • Resolving these outstanding issues in XLA is a priority due to its prevalence among IEIs.
  • Future research should focus on understanding and addressing these complex aspects of XLA.
  • Exploring novel therapeutic strategies, including stem cell transplantation, may improve patient outcomes.