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Semin Hematol. 2002 Apr;39(2):107-12.

Neutropenia associated with primary immunodeficiency syndromes.

Author information

1
Department of Pediatrics, Cancercare Manitoba, University of Manitoba, Winnipeg, Canada.

Abstract

The primary immunodeficiency diseases are a heterogeneous group of more than 75 disorders characterized by intrinsic defects in the functions of the immune system. Many are associated with abnormalities of hematopoiesis as well. This article will review those primary immunodeficiency syndromes in which neutropenia is a prominent finding, including X-linked agammaglobulinemia (XLA), hyper IgM syndrome, common variable immunodeficiency (CVID), IgA deficiency, cartilage-hair hypoplasia (CHH), and reticular dysgenesis, with regards to pathophysiologic findings and treatment.

PMID:
11957193
DOI:
10.1053/shem.2002.31916
[Indexed for MEDLINE]

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