Clinical Working Party

Common Variable Immunodeficiency (CVI) diagnosis criteria

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Probable

Male or female patient who has a marked decrease of IgG (at least 2 SD below the mean for age) and a marked decrease in at least one of the isotypes IgM or 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 (see 'Differential Diagnosis of Hypogammaglobulinemia')

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 (see 'Differential Diagnosis of Hypogammaglobulinemia')

Spectrum of disease

Most patients with CVI are recognized to have immunodeficiency in the second, third or fourth decade of life, after they have had several pneumonias; however children and older adults may be affected. Viral, fungal and parasitic infections as well as bacterial infections may be problematic. The serum concentration of IgM is normal in about half of the patients. Abnormalities in T cell numbers or function are common. The majority of patients have normal numbers of B cells; however, some have low or absent B cells. Approximately 50% of patients have autoimmune manifestations. There is an increased risk of malignancy.