Diagnostic Criteria for Common Variable Immunodeficiency (CVI)

Common variable immunodeficiency (CVID) is a rare immune deficiency characterized by low levels of serum IgG, IgA, and/or IgM, with a loss of Ab production. The diagnosis is most commonly made in adults between the ages of 20 and 40 years, but both children and much older adults can be found to have this immune defect. 

Diagnostic Criteria

Probable

Male or female patient who has a marked decrease (at least 2 SD below the mean for age) in serum IgG and IgA and fulfills all of the following criteria:

  1. Onset of immunodeficiency at greater than 2 years of age.

  2. Absent isohemagglutinins and/or poor response to vaccines.

  3. Defined causes of hypogammaglobulinemia have been excluded.

Possible

Male or female patient who has a marked decrease (at least 2 SD below the mean for age) in one of the major isotypes (IgM, IgG, and IgA) and fulfills all of the following criteria:

  1. Onset of immunodeficiency at greater than 2 years of age.

  2. Absent isohemagglutinins and/or poor response to vaccines.

  3. Defined causes of hypogammaglobulinemia have been excluded.

Differential Diagnosis of Hypogammaglobulinemia

Drug induced

  • Antimalarial agents

  • Captopril

  • Carbamazepine

  • Glucocorticoids

  • Fenclofenac

  • Gold salts

  • Penicillamine

  • Phenytoin

  • Sulfasalazine

Genetic disorders

  • Ataxia telangiectasia

  • Autosomal forms of Severe Combined Immunodeficiency (SCID)

  • Hyper IgM immunodeficiency

  • Transcobalamin II deficiency and hypogammaglobulinemia

  • X-linked agammaglobulinemia

  • X-linked lymphoproliferative disorder (EBV associated)

  • X-linked SCID

  • Some metabolic disorders

Chromosomal anomalies

  • Chromosome 18q2 syndrome

  • Monosomy 22

  • Trisomy 8

  • Trisomy 21

Infectious diseases

  • HIV

  • Congenital rubella

  • Congenital infection with CMV

  • Congenital infection with Toxoplasma gondii

  • Epstein–Barr virus

Malignancy

  • Chronic lymphocytic leukemia

  • Immunodeficiency with thymoma

  • Non-Hodgkin lymphoma

  • B cell malignancy

Systemic disorders

  • Immunodeficiency caused by hypercatabolism of immunoglobulin

  • Immunodeficiency caused by excessive loss of immunoglobulins (nephrosis, severe burns, lymphangiectasia, severe diarrhea)

 

 

References:

  1. Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol. 1999 Dec;93(3):190-7. [Medline]

Created: Nov 03, 2007