Report of the National Immunoglobulin Replacement Expert Committee: algorithm for diagnosis of immunodeficiency requiring antibody replacement therapy

Immunoglobulin replacement therapy is a mainstay in the treatment of immune deficiencies characterized by antibody failure. Whether the cause is primary or secondary, affected patients frequently present with a history recurrent and complicated infections of the upper and (or) lower respiratory tract. Such replacement therapy has been available since the 1980s, although treatment modalities have since been refined to provide improved protection against infections resulting in reduced morbidity and mortality. Here, we describe an algorithm for diagnosing patients with suspected primary or secondary immunodeficiency, including assessment of clinical, laboratory, and genetic information, when considering initiating immunoglobulin replacement. The increasing availability of molecular genetic techniques will likely result in decreased diagnostic delay for these patients. Statement of novelty: We describe here an algorithm for diagnosing patients with immunodeficiency requiring immunoglobulin replacement therapy.