Neutropenia associated with primary immunodeficiency syndromes.
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.
Cham
Bonnie
B
Department of Pediatrics, Cancercare Manitoba, University of Manitoba, Winnipeg, Canada.
Bonilla
Mary Ann
MA
Winkelstein
Jerry
J
D000361:Agammaglobulinemia; D017074:Common Variable Immunodeficiency; D006410:Hematopoiesis; D006801:Humans; D017098:IgA Deficiency; D007075:Immunoglobulin M; D007153:Immunologic Deficiency Syndromes / Q000150:complications / Q000473:pathology* / Q000628:therapy; D009503:Neutropenia / Q000209:etiology*
D007075:Immunoglobulin M