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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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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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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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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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B Cell Activation and Differentiation01:24

B Cell Activation and Differentiation

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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...
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Cell-mediated Immune Responses01:40

Cell-mediated Immune Responses

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Overview
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Orbital diffuse large B-cell lymphoma with combined variable immunodeficiency.

Vishal S Parikh1, Deepa Jagadeesh2, James M Fernandez3

  • 1a Department of Ophthalmology, Cole Eye Institute , Cleveland Clinic Foundation , Cleveland , Ohio , USA.

Orbit (Amsterdam, Netherlands)
|July 19, 2017
PubMed
Summary
This summary is machine-generated.

Common variable immunodeficiency (CVID) patients face an increased lymphoma risk. Early diagnosis of orbital masses in CVID is crucial for treating aggressive diffuse large B-cell lymphoma (DLBCL) and achieving good prognoses.

Keywords:
Combined variablediffuse large B-cell lymphomaimmunodeficiencyorbital lymphoma

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Area of Science:

  • Immunology
  • Oncology
  • Ophthalmology

Background:

  • Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by low immunoglobulin levels and increased infection susceptibility.
  • CVID is associated with a higher risk of developing malignancies, particularly lymphoma.

Observation:

  • A 63-year-old female with CVID presented with orbital symptoms including pain, proptosis, and eyelid fullness.
  • MRI revealed a diffuse orbital mass involving muscles and the optic nerve.
  • Biopsy suggested a large B-cell neoplasm, later confirmed as diffuse large B-cell lymphoma (DLBCL) via PET-guided lymph node biopsy.

Findings:

  • The patient received chemotherapy and radiation therapy for DLBCL.
  • Treatment resulted in minimal residual disease on PET scan and complete remission.

Implications:

  • Orbital masses in patients with primary immunodeficiencies, like CVID, can represent aggressive malignancies such as DLBCL.
  • Prompt diagnosis and systemic treatment are essential for a favorable prognosis in these cases.