WAS is a rare inherited disease characterized by immune dysregulation and microthrombocytopenia. Diagnostic criteria for WAS has been
published by the Pan-American Group for Immunodeficiency. Probable criteria include a male patient with thrombocytopenia (<70 × 103/mm3), low mean platelet volume (< 5.0 fL), and at least 1 of the following: eczema; low level of isohemagglutinins; poor vaccine responses to polysaccharide antigens, recurrent bacterial, viral or opportunistic infections; autoimmune disease; and lymphoreticular malignancy. Many patients with WAS have low levels of IgM and increased levels of IgA, IgD, and IgE.
1. Bonilla FA, Geha RS. 12. Primary immunodeficiency diseases [published erratum appears in J Allergy Clin Immunol 2003;112:267]. J Allergy Clin Immunol 2003;111(2 Suppl):S571-81.
2. Mouthon L, Lortholary O. Intravenous immunoglobulins in infectious diseases: where do we stand? Clin Microbiol Infect 2003;9:333-8.
3. Tangsinmankong N, Bahna SL, Good RA. The immunologic workup of the child suspected of immunodeficiency. Ann Allergy Asthma Immunol 2001;87:362-9.
Click here to return to the questions
Seminars in Medical Practice
Hospital Physician Board Review Manuals
Copyright © 2009, Turner White Communications
Updated 1/04/08 kkj