Plasmacytoid dendritic cell depletion in DOCK8 deficiency: Rescue of severe herpetic infections with IFN-α 2b therapy Sevgi Keles, MD, Haifa H. Jabara,

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Plasmacytoid dendritic cell depletion in DOCK8 deficiency: Rescue of severe herpetic infections with IFN-α 2b therapy Sevgi Keles, MD, Haifa H. Jabara, BSc, Ismail Reisli, MD, Douglas R. McDonald, MD, PhD, Isil Barlan, MD, Rima Hanna-Wakim, MD, Ghassan Dbaibo, MD, Gerard Lefranc, PhD, Waleed Al-Herz, MD, Raif S. Geha, MD, Talal A. Chatila, MD, MSc Journal of Allergy and Clinical Immunology Volume 133, Issue 6, Pages 1753-1755.e3 (June 2014) DOI: 10.1016/j.jaci.2014.03.032 Copyright © 2014 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Representative pictures of the lesions in patients (Pt) 1 and 2 early in their infection (A and D), just before IFN-α 2b therapy (B and E) and 6 months into IFN-α 2b therapy (C and F), respectively. Journal of Allergy and Clinical Immunology 2014 133, 1753-1755.e3DOI: (10.1016/j.jaci.2014.03.032) Copyright © 2014 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 2 A, Representative FACS analysis for BDCA-4+ CD123+ pDCs in the lymphocyte gate of PBMCs from a control and a DOCK8-deficient subject. B, Total number of pDCs/106 cells in controls (C, n = 10) and patients (Pt, n = 9). C, IFN-α production in supernatants of CpG-stimulated PBMCs from controls (n = 9) and patients (n = 8). D, Production of IFN-α/pDC in controls and patients (n = 7 each). *P < .05. FACS, Fluorescence-activated cell sorting; ns, nonsignificant. Journal of Allergy and Clinical Immunology 2014 133, 1753-1755.e3DOI: (10.1016/j.jaci.2014.03.032) Copyright © 2014 American Academy of Allergy, Asthma & Immunology Terms and Conditions