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Clinical Microbiology Reviews, July 2009, p. 396-414, Vol. 22, No. 3
0893-8512/09/$08.00+0     doi:10.1128/CMR.00001-09
Copyright © 2009, American Society for Microbiology. All Rights Reserved.

Pathogenesis, Diagnosis, and Management of Primary Antibody Deficiencies and Infections

Ari J. Fried* and Francisco A. Bonilla Author Bios

Division of Immunology, Children's Hospital Boston, Boston, Massachusetts 02115, and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115

Summary: Primary antibody deficiencies are the most common primary immunodeficiency diseases. They are a heterogeneous group of disorders with various degrees of dysfunctional antibody production resulting from a disruption of B-cell differentiation at different stages. While there has been tremendous recent progress in the understanding of some of these disorders, the etiology remains unknown for the majority of patients. As there is a large spectrum of underlying defects, the age at presentation varies widely, and the clinical manifestations range from an almost complete absence of B cells and serum immunoglobulins to selectively impaired antibody responses to specific antigens with normal total serum immunoglobulin concentrations. However, all of these disorders share an increased susceptibility to infections, affecting predominantly the respiratory tract. A delay of appropriate treatment for some diseases can result in serious complications related to infections, while timely diagnosis and adequate therapy can significantly decrease morbidity and increase life expectancy and quality of life.

* Corresponding author. Mailing address: Children's Hospital Boston, Fegan Building, 6th Floor, 300 Longwood Avenue, Boston, MA 02115. Phone: (617) 355-6117. Fax: (617) 730-0310. E-mail: ari.fried{at}childrens.harvard.edu

Clinical Microbiology Reviews, July 2009, p. 396-414, Vol. 22, No. 3
0893-8512/09/$08.00+0     doi:10.1128/CMR.00001-09
Copyright © 2009, American Society for Microbiology. All Rights Reserved.





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