Diagnostic Criteria for Common Variable Immunodeficiency (CVI)

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)

Created: Nov 03, 2007
Last Modified: 11/03/2007