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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...
919

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Isolation of Brain-infiltrating Leukocytes
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Granulomatous Brain Involvement in Common Variable Immunodeficiency: A Case Report.

Maria Rocha1, Rita Gouveia1, Ana Neves1

  • 1Internal Medicine, Centro Hospitalar Universitário de São João, Porto, PRT.

Cureus
|September 26, 2024
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Summary

Common variable immunodeficiency (CVID) can cause rare brain lesions, even without typical infections. Early diagnosis and treatment with immunoglobulin therapy improved patient outcomes.

Keywords:
autoimmunitycase reportcommon variable immunodeficiencygranulomatous-lymphocytic interstitial diseaseinfiltrative brain lesions

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Area of Science:

  • Immunology
  • Neurology

Background:

  • Common variable immunodeficiency (CVID) is a primary immunodeficiency characterized by impaired B cell differentiation and defective immunoglobulin production.
  • CVID often presents with recurrent infections, autoimmune diseases, and inflammatory disorders, potentially delaying diagnosis.

Observation:

  • A woman in her 30s with a history of Evans syndrome and lymphocytic alveolitis presented with persistent headache and multiple infiltrative brain lesions on imaging.
  • The patient lacked typical recurrent infections but had a history of autoimmune manifestations and immunoglobulin deficiencies.

Findings:

  • The clinical presentation and history led to a diagnosis of CVID without requiring a brain biopsy.
  • Treatment with intravenous immunoglobulin and immunosuppressive therapy resulted in significant clinical improvement and resolution of brain lesions.

Implications:

  • This case underscores the importance of considering CVID in patients with autoimmune manifestations, even in the absence of recurrent infections.
  • Prompt immunoglobulin replacement and immunosuppression are effective in managing severe CVID presentations, including rare neurological involvement.