Monitoring

Patients with hypogammaglobulinemia requiring immunoglobulin replacement should be monitored clinically for infection rates, infective complications, malignancy, and other complications. There is no published consensus on the ideal frequency of monitoring tests, but the following is generally used:

  • CBC, liver function tests, and IgG trough levels measured at least every 3 to 6 months

  • Viral hepatitis screening (HBsAg, hepatitis B and C polymerase chain reaction) and lymphocyte subsets performed annually

  • Serum saved annually or with any change of immunoglobulin product

  • Pulmonary function tests performed at least annually

  • Regular CT scans of the chest (with fewer cuts as appropriate); frequency of scanning as clinically indicated, and balanced against lifetime radiation risk as some primary immunodeficiencies are radiosensitive (e.g., ataxia-telangiectasia, common variable immunodeficiency)[69][70]

  • Other investigations performed as needed.

Patients with milder forms of hypogammaglobulinemia (e.g., IgA deficiency, impaired specific antibody deficiency) who do not require immunoglobulin replacement require monitoring of symptoms and immunoglobulin levels to ensure disease is not progressing.

There are increasing reports of prolonged and symptomatic hypogammaglobulinemia following certain treatments, including biologics such as rituximab and other B-cell targeted therapies.[20][21][22][23][55][56] Patients receiving rituximab should have their immunoglobulin levels monitored regularly (e.g., every 6 months).[22][23][57][58][82] 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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