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Related Concept Videos

Cells of the Adaptive Immune Response01:23

Cells of the Adaptive Immune Response

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...
Special Features of Adaptive Immunity01:20

Special Features of Adaptive Immunity

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.
The primary cell types involved in adaptive immunity are T cells and B cells. Each type has a unique role in defending the body against pathogens. T cells are responsible for cell-mediated immunity. They identify and eliminate infected cells directly,...
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...
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...
Cell-mediated Immune Responses01:40

Cell-mediated Immune Responses

Overview
Introduction to Innate and Adaptive Immunity01:21

Introduction to Innate and Adaptive Immunity

The human immune system is a complex defense mechanism that protects the body from harmful pathogens and foreign substances. It comprises two crucial components: innate and adaptive immunity.
Innate immunity is the body's natural, nonspecific defense system that acts quickly to protect against pathogens. It incorporates physical barriers like skin and mucous membranes and cellular elements such as phagocytes and natural killer cells. This part of our immune system provides an immediate,...

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Intersection Between Systemic Autoimmune Diseases, Primary Immunodeficiency and Cancer: a Field in its Infancy.

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Exploring the Burden on Patients Living With and Receiving Treatment for Immune Thrombocytopenia (ITP): Patient and Physician Perceptions From the ITP World Impact Survey (I-WISh) 2.0.

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From variants to answers: The evolution of genetic counseling in IEI.

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Fetal and neonatal alloimmune thrombocytopenia (FNAIT): Survey of UK fetal medicine centres on antenatal management of subsequent affected pregnancies.

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Navigating primary and secondary immunodeficiency intersections: how to find IEI hidden within SID.

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Chimeric antigen receptor T cells for autoimmune diseases, in particular immune thrombocytopenia.

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Related Experiment Video

Updated: Jun 28, 2026

Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity
11:12

Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity

Published on: April 11, 2019

In a nutshell: Pandora's Box-B-cell lymphoproliferation masking inborn errors of immunity.

Silvia Sánchez-Ramón1,2, James B Bussel3

  • 1Department of Clinical Immunology, Institute of Laboratory Medicine and IdISSC, Hospital Clínico San Carlos, Madrid, Spain.

British Journal of Haematology
|June 27, 2026
PubMed
Summary

Some patients with B-cell lymphoproliferative disorders (B-CLPDs) and secondary immunodeficiency (SID) may have hidden primary immunodeficiencies (PIDs). Identifying these PIDs reveals genetic links to cancer and guides personalized treatment.

Keywords:
B‐cell lymphoproliferative disordersinborn errors of immunitysecondary immunodeficiency

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

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Interrogating Individual Autoreactive Germinal Centers by Photoactivation in a Mixed Chimeric Model of Autoimmunity
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The bm12 Inducible Model of Systemic Lupus Erythematosus (SLE) in C57BL/6 Mice
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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

Area of Science:

  • Immunology
  • Oncology
  • Genetics

Background:

  • Immunodeficiency is a significant risk factor for cancer, especially in B-cell lymphoproliferative disorders (B-CLPDs).
  • Patients with B-CLPDs and secondary immunodeficiency (SID) may have undiagnosed primary immunodeficiencies (PIDs).
  • Recognizing these hidden PIDs is crucial for accurate classification and understanding cancer-associated immunodeficiency.

Purpose of the Study:

  • To identify patients with B-CLPDs who have underlying PIDs that were initially misclassified as SID.
  • To explore the genetic basis of cancer-associated immunodeficiency by analyzing tumor somatic variants.
  • To advance precision oncohaematology through a host-centered approach.

Main Methods:

  • Analysis of patients with B-CLPDs initially presenting with SID.
  • Identification of tumor somatic variants.
  • Comparison of somatic variants with germline genes known to cause PIDs.

Main Results:

  • Discovery of overlapping tumor somatic variants and PID-causative germline genes.
  • Uncovering novel mechanisms of immune dysfunction in B-CLPD.
  • Establishing a link between specific genetic variants and increased cancer risk.

Conclusions:

  • Identifying hidden PIDs in B-CLPD patients refines disease classification and broadens understanding of cancer-associated immunodeficiency.
  • Discovering shared genetic variants offers new targets for precision oncohaematology.
  • An individualized, host-centered approach improves patient care, early detection, and long-term outcomes.