Early-onset monocyte–B–natural killer–dendritic cells’ deficiency successfully treated with hematopoietic stem cell transplantation Caterina Cancrini,

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Presentation transcript:

Early-onset monocyte–B–natural killer–dendritic cells’ deficiency successfully treated with hematopoietic stem cell transplantation Caterina Cancrini, MD, PhD, Alessia Scarselli, MD, Samantha Scaramuzza, BSc, PhD, Maria Chiriaco, BSc, PhD, Silvia Di Cesare, BSc, Gigliola Di Matteo, BSc, PhD, Maria Luisa Romiti, BSc, PhD, Paolo Palma, MD, PhD, Lidia De Felice, BSc, Giuseppe Palumbo, MD, PhD, Rita Maria Pinto, MD, Rita De Vito, MD, Luigi Racioppi, MD, PhD, Susanna Livadiotti, MD, Alain Fischer, MD, PhD, Paolo Rossi, MD, PhD, Maurizio Caniglia, MD, Alessandro Aiuti, MD, PhD Journal of Allergy and Clinical Immunology Volume 128, Issue 4, Pages 897-900.e1 (October 2011) DOI: 10.1016/j.jaci.2011.07.044 Copyright © 2011 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Immune phenotype and functional assays of PB and BM. A, Flow cytometry of myeloid and plasmacytoid dendritic cells in the patient’s PB before and after (1 year) HSCT as compared with a healthy donor (HD). Gate A is drawn in order to exclude granulocytes, CD19+ B cells, and monocytes (CD14+). B, BM morphology and clonogenic assays (with SCF, GM-CSF, IL-3, and EPO). Normal values of colonies/100,000 mononuclear BM cells: CFU-GM: 155 ± 105, BFU-E: 159 ± 73, CFU-GEMM: 38 ± 23. C, In vitro BM CD34+ cell differentiation in B and NK cells with stromal cells and cytokines (IL-2, IL-7, SCF, IL-15, TPO, FLT3 ligand). Graphs show representative wells containing differentiated B, NK, or myeloid cells after 3 weeks of culture. The table shows the proportion of wells scoring positive for the specific marker. D, In vitro proliferation responses of BM CD34+ cells to different cytokines that are measured by thymidine incorporation. Pt, Patient. Journal of Allergy and Clinical Immunology 2011 128, 897-900.e1DOI: (10.1016/j.jaci.2011.07.044) Copyright © 2011 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E1 Flow cytometry of BM mononuclear cells for the expression of CD19, CD117 (c-kit), CD127 (IL7-R), and CXCR4 on CD34+ cells before and after bone marrow transplantation (BMT) in comparison to a healthy donor. n.d., Not done. Journal of Allergy and Clinical Immunology 2011 128, 897-900.e1DOI: (10.1016/j.jaci.2011.07.044) Copyright © 2011 American Academy of Allergy, Asthma & Immunology Terms and Conditions