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They most commonly present with recurrent infections due to encapsulated bacteria, although in the most common antibody deficiency, Common Variable Immunodeficiency, systemic and organ-specific autoimmunity can be a presenting feature. Diagnostic delay in this group of disorders remains a problem, and the laboratory has a vital role in the detection of abnormalities in immunoglobulin concentration and function. It is critical to distinguish this group of disorders from secondary causes of hypogammaglobulinaemia, in particular lymphoid malignancy, and appropriate laboratory investigations are of critical importance. Treatment of primary antibody deficiencies involves immunoglobulin replacement therapy, either via the intravenous or subcutaneous route. Patients remain at risk of a wide variety of complications, not all linked to diagnostic delay and inadequate therapy. In common variable immunodeficiency (CVID) in particular, patients remain at significantly increased risk of lymphoid malignancy, and regular clinical and laboratory monitoring is required. This review aims to give an overview of these conditions for the general reader, covering pathogenesis, clinical presentation, laboratory investigation, therapy and clinical management.<\/jats:p>","DOI":"10.1258\/acb.2008.008175","type":"journal-article","created":{"date-parts":[[2009,2,18]],"date-time":"2009-02-18T10:17:55Z","timestamp":1234952275000},"page":"99-108","source":"Crossref","is-referenced-by-count":23,"title":["Primary antibody deficiency syndromes"],"prefix":"10.1177","volume":"46","author":[{"given":"P","family":"Wood","sequence":"first","affiliation":[{"name":"Clinical Immunology Unit, St James's University Hospital, Leeds LS9 7TF, 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