Secondary prevention

Relatives of patients with known genetic mutations causing hypogammaglobulinemia should be offered counseling followed by genetic testing if desired. Preimplantation genetic diagnosis has been offered in several cases where parents are known carriers. Screening of immunoglobulin levels in relatives of common variable immunodeficiency (CVID) patients demonstrates familial inheritance in 10% to 20% of CVID and IgA deficiency cases.[84]

Secondary prevention also involves carefully monitoring patients likely to develop secondary hypogammaglobulinemia: for example, measuring immunoglobulins in patients with hematologic malignancy, or in those who are receiving chemotherapy or immunosuppressive therapy (e.g., rituximab). Rituximab is a B-cell depleting agent used in the treatment of hematologic malignancy and autoimmune disease. There is increasing recognition of rituximab-induced hypogammaglobulinemia usually following multiple doses of rituximab.[20][21][22][23] Immunoglobulin levels should be monitored regularly (e.g., every 6 months) in patients receiving rituximab.[22][23][57][58] Additionally, there is increasing data that pediatric patients treated with rituximab may be at higher risk for hypogammaglobulinemia and should be monitored.[23][58]

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