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Science 3 August 2007:
Vol. 317. no. 5838, pp. 617 - 619
DOI: 10.1126/science.1142963
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Perspective

Primary Immunodeficiencies: A Field in Its Infancy

Jean-Laurent Casanova1,2,3* and Laurent Abel1,2

A paradigm shift is occurring in the field of primary immunodeficiencies, with revision of the definition of these conditions and a considerable expansion of their limits. Inborn errors of immunity were initially thought to be confined to a few rare, familial, monogenic, recessive traits impairing the development or function of one or several leukocyte subsets and resulting in multiple, recurrent, opportunistic, and fatal infections in infancy. A growing number of exceptions to each of these conventional qualifications have gradually accumulated. It now appears that most individuals suffer from at least one of a multitude of primary immunodeficiencies, the dissection of which is helping to improve human medicine while describing immunity in natura.

1 Laboratory of Human Genetics of Infectious Diseases, Institut National de la Santé et de la Recherche Médicale, U550, Paris, France.
2 University Paris René Descartes, Necker Medical School, Paris, France.
3 Pediatric Hematology and Immunology Unit, Necker Children's Hospital, Paris, France.

* To whom correspondence should be addressed. E-mail: casanova{at}necker.fr

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Science. ISSN 0036-8075 (print), 1095-9203 (online)