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Allergy/Immunology
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Answer 2
- WAS.
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.
SUGGESTED READINGS
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.
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