Broad spectrum of autoantibodies in patients with Wiskott-Aldrich syndrome and X- linked thrombocytopenia Elena Crestani, MD, Stefano Volpi, MD, Fabio.

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Presentation transcript:

Broad spectrum of autoantibodies in patients with Wiskott-Aldrich syndrome and X- linked thrombocytopenia Elena Crestani, MD, Stefano Volpi, MD, Fabio Candotti, MD, Silvia Giliani, PhD, Lucia Dora Notarangelo, MD, Julia Chu, MD, Juan Carlos Aldave Becerra, MD, David Buchbinder, MD, Janet Chou, MD, Raif S. Geha, MD, Maria Kanariou, MD, Alejandra King, MD, Cinzia Mazza, PhD, Daniele Moratto, PhD, Robert Sokolic, MD, Elizabeth Garabedian, RN, MSLS, Fulvio Porta, MD, Maria Caterina Putti, MD, Rima H. Wakim, MD, Erdyni Tsitsikov, PhD, Sung-Yun Pai, MD, Luigi D. Notarangelo, MD Journal of Allergy and Clinical Immunology Volume 136, Issue 5, Pages 1401-1404.e3 (November 2015) DOI: 10.1016/j.jaci.2015.08.010 Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 A and B, Heat map of IgG (Fig 1, A) and IgA (Fig 1, B) autoantibodies in patients with XLT and those with WAS. For each self-antigen, a colorimetric representation of RAR in each sample is shown according to the scale depicted at the top. C and D, Autoantibodies with significantly increased reactivity in patients with WAS and those with XLT, respectively, compared with those in healthy donors (HD). Journal of Allergy and Clinical Immunology 2015 136, 1401-1404.e3DOI: (10.1016/j.jaci.2015.08.010) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E1 Percentage of self-antigens for which there were positive IgG (A) and IgA (B) autoantibody levels. Five hundred eighty autoantibodies were demonstrated in patients with WAS and those with XLT. Journal of Allergy and Clinical Immunology 2015 136, 1401-1404.e3DOI: (10.1016/j.jaci.2015.08.010) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E2 IgG (A) and IgA (B) serum levels in multireactive and paucireactive patients with WAS/XLT. Journal of Allergy and Clinical Immunology 2015 136, 1401-1404.e3DOI: (10.1016/j.jaci.2015.08.010) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E3 A and B, Frequency of positivity for the 25 most common autoantibodies. C, RAR values for 2 IgG autoantibodies (to fibrinogen IV and mitochondrial antigen) with significantly increased levels in patients with XLT and those with WAS compared with levels in healthy control subjects (HD). Journal of Allergy and Clinical Immunology 2015 136, 1401-1404.e3DOI: (10.1016/j.jaci.2015.08.010) Copyright © 2015 American Academy of Allergy, Asthma & Immunology Terms and Conditions