《Antibody deficiency diseases》.pdf

Eur. J. Immunol. 2008. 38: 327–333 Highlights 327 Mini-Review: Antibody deficiency diseases Qiang Pan-Hammarstrm and Lennart Hammarstrm Div. of Clinical Immunology, Dept. of Laboratory Medicine, Karolinska University Received 17/10/07 Hospital Huddinge, Karolinska Institutet, Stockholm, Sweden Revised 21/11/07 Accepted 12/12/07 Primary immunodeficiency diseases are rare disorders characterized by quantitative or [DOI 10.1002/eji.200737927] qualitative defects in cells or components in the immune system, resulting in a high Key words: degree of susceptibility to various types of infections. During differentiation, stem cells Antibody Common undergo a series of discrete steps, governed by a large number of different genes. variable immunodefi- Mutations/deletions in these genes will result in a block in differentiation of the affected ciency IgA deficiency cell lineage(s), leading to immunodeficiency. To date, more than 150 different types of Primary immuno- disorders have been described. In this review, we will focus on novel findings in