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Common Variable Deficiency
&
Selective IGE Deficiency
Presented by:
Saqib Ali Shah
Introduction
• Common variable immunodeficiency (CVID)
• First, defined in 1971 by WHO as separate less well- defined antibody
deficiency syndromes from others with a more coherent clinical
description and Mendelian inheritance.
• Most common and clinically most important severe primary antibody
deficiency.
• Heterogenous disease
What is CVID
• Common variable immunodeficiency (CVID) is a disorder that involves
the following:
• Low levels of most or all of the immunoglobulin (Ig) classes
• A lack of B lymphocytes or plasma cells that are capable of producing
antibodies
• Frequent bacterial infections.
• The World Health Organization (WHO) recognizes more than 150
primary immunodeficiency's ranging from relatively common to quite
rare.
Pathogenesis
• Loss of B cell function
• T and B cell dysregulation
• Defective antibody production
Infections in CVID
• Respiratory : upper and lower Gastrointestinal
• Genitourinary tracts : Ureaplasma urealyticum
• Arthritis : Ureaplasma or Mycoplasma
Diagnosis of CVID
Persistent drop in immunoglobulin level ( < 2 SD) for 2 years since onset
along with all 3 criteria’s
Minimum Criteria
• Onset of immunodeficiency > 4 Y age
• Decrease response to vaccines
• Exclude other causes of immunodeficiency IgG and IgA
Probable Diagnosis IgG, IgA
Possible Diagnosis IgG ,M, A
• The number of T-lymphocytes may also be determined and their
function tested in samples of blood.
• With special laboratory techniques, it is possible to determine if B-
lymphocytes produce antibody in a test tube (tissue culture) and if
T-lymphocytes have normal functions.
Selective IgE deficiency (IgED)
• Selective IgE deficiency (IgED) is currently defined as a
significant reduction in serum levels of IgE (less than or
equal to 2 kIU/L) in a patient whose other immunoglobulin
levels are normal or diminished.
Selective IgE deficiency (IgED)
• Patients with IgE hypogammaglobulinemia were found to
have an increased prevalence of multiple immunoglobulin
deficits, autoimmune disease, and nonallergic reactive
airway disease.
Continue..
• IgE is thought to be potentially atherogenic through its
proinflammatory influence on mast cells and platelets
Saqib PPT.pptx

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Saqib PPT.pptx

  • 1. Common Variable Deficiency & Selective IGE Deficiency Presented by: Saqib Ali Shah
  • 2. Introduction • Common variable immunodeficiency (CVID) • First, defined in 1971 by WHO as separate less well- defined antibody deficiency syndromes from others with a more coherent clinical description and Mendelian inheritance. • Most common and clinically most important severe primary antibody deficiency. • Heterogenous disease
  • 3.
  • 4. What is CVID • Common variable immunodeficiency (CVID) is a disorder that involves the following: • Low levels of most or all of the immunoglobulin (Ig) classes • A lack of B lymphocytes or plasma cells that are capable of producing antibodies • Frequent bacterial infections. • The World Health Organization (WHO) recognizes more than 150 primary immunodeficiency's ranging from relatively common to quite rare.
  • 5. Pathogenesis • Loss of B cell function • T and B cell dysregulation • Defective antibody production
  • 6. Infections in CVID • Respiratory : upper and lower Gastrointestinal • Genitourinary tracts : Ureaplasma urealyticum • Arthritis : Ureaplasma or Mycoplasma
  • 7. Diagnosis of CVID Persistent drop in immunoglobulin level ( < 2 SD) for 2 years since onset along with all 3 criteria’s Minimum Criteria • Onset of immunodeficiency > 4 Y age • Decrease response to vaccines • Exclude other causes of immunodeficiency IgG and IgA Probable Diagnosis IgG, IgA Possible Diagnosis IgG ,M, A
  • 8. • The number of T-lymphocytes may also be determined and their function tested in samples of blood. • With special laboratory techniques, it is possible to determine if B- lymphocytes produce antibody in a test tube (tissue culture) and if T-lymphocytes have normal functions.
  • 9. Selective IgE deficiency (IgED) • Selective IgE deficiency (IgED) is currently defined as a significant reduction in serum levels of IgE (less than or equal to 2 kIU/L) in a patient whose other immunoglobulin levels are normal or diminished.
  • 10. Selective IgE deficiency (IgED) • Patients with IgE hypogammaglobulinemia were found to have an increased prevalence of multiple immunoglobulin deficits, autoimmune disease, and nonallergic reactive airway disease.
  • 11. Continue.. • IgE is thought to be potentially atherogenic through its proinflammatory influence on mast cells and platelets