Timely follow-up of a GATA2 deficiency patient allows successful treatment Sara Ciullini Mannurita, MSc, Marina Vignoli, PhD, Gloria Colarusso, MD, PhD,

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Timely follow-up of a GATA2 deficiency patient allows successful treatment Sara Ciullini Mannurita, MSc, Marina Vignoli, PhD, Gloria Colarusso, MD, PhD, Fabio Tucci, MD, Marinella Veltroni, MD, Stefano Frenos, MD, Veronica Tintori, MD, Maurizio Aricò, MD, Venetia Bigley, MD, PhD, Matthew Collin, MD, PhD, Claudio Favre, MD, Eleonora Gambineri, MD Journal of Allergy and Clinical Immunology Volume 138, Issue 5, Pages 1480-1483.e4 (November 2016) DOI: 10.1016/j.jaci.2016.06.004 Copyright © 2016 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Flow cytometry analysis of peripheral blood cells showing the identification of monocyte and dendritic cell subsets in a patient and a healthy subject. Gating strategy and cell populations are shown in the healthy subject dot plots. The absence of pDC and mDC and decrease in monocytes are evident in the patient compared with the healthy subject. mDC, Myeloid dendritic cell; pDC, plasmacytoid dendritic cell. Journal of Allergy and Clinical Immunology 2016 138, 1480-1483.e4DOI: (10.1016/j.jaci.2016.06.004) Copyright © 2016 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 2 Immune reconstitution after HSCT. Time zero (x axis) represents the date of HSCT. A, Absolute count of T, B, and NK cells (per μL of blood) in peripheral blood pretransplant and posttransplant. B, Dendritic cell immune reconstitution at different times posttransplant. The increase in mDC and pDC subpopulations is evident. Gating strategy for the identification of human pDC and mDC is shown in the lower panel. HSCT, Hematopoietic stem cell transplantation; mDC, myeloid dendritic cell; NK, natural killer; pDC, plasmacytoid dendritic cell. Journal of Allergy and Clinical Immunology 2016 138, 1480-1483.e4DOI: (10.1016/j.jaci.2016.06.004) Copyright © 2016 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E1 Bone marrow aspirate smear stained with May-Grünwald Giemsa (original magnification ×100). Presence of cytoplasmic bridges between 2 erythroblasts (A and B), binuclear erythroblast (C), giant platelets (arrow) (D). Journal of Allergy and Clinical Immunology 2016 138, 1480-1483.e4DOI: (10.1016/j.jaci.2016.06.004) Copyright © 2016 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E2 Timeline representing HSCT conditioning and GvHD prophylaxis. The numbered line indicates the time (days) before and after transplantation. Day zero is the day of graft infusion. CsA, Cyclosporin A; GvHD, graft-versus-host disease; HSCT, hematopoietic stem cell transplantation; MNC, mononuclear cells; MTX, methotrexate. Journal of Allergy and Clinical Immunology 2016 138, 1480-1483.e4DOI: (10.1016/j.jaci.2016.06.004) Copyright © 2016 American Academy of Allergy, Asthma & Immunology Terms and Conditions