Predominantly antibody deficiencies

Abstract

Antibody deficiencies are the most common forms of primary immunodeficiency diseases, generally easily controlled by immunoglobulin substitution. Some of antibody deficiencies could be secondary to a T cell deficiency that has to be intensively looked for, since it severely worsens the prognosis and modifies the treatment. In the absence of early diagnosis and appropriate management, primary antibody deficiency can lead to serious morbidity and early mortality. A diagnostic delay in affected patients with primary antibody deficiency still remains a significant problem due to limited awareness of their presenting features. Clinical history is the most important aspect of suspecting a diagnosis of primary antibody deficiency. Defining the molecular defect of antibody deficiencies is essential for accurate diagnosis, including prenatal diagnosis, and appropriate management of patients. © 2008 Springer Berlin Heidelberg.