Allogeneic transplantation successfully corrects immune defects, but not susceptibility to colitis, in a patient with nuclear factor-κB essential modulator.

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Presentation transcript:

Allogeneic transplantation successfully corrects immune defects, but not susceptibility to colitis, in a patient with nuclear factor-κB essential modulator deficiency Sung-Yun Pai, MD, Ofer Levy, MD, PhD, Haifa H. Jabara, BS, Jonathan N. Glickman, MD, PhD, Liat Stoler-Barak, MS, Jessica Sachs, MD, Samuel Nurko, MD, Jordan S. Orange, MD, PhD, Raif S. Geha, MD Journal of Allergy and Clinical Immunology Volume 122, Issue 6, Pages 1113-1118.e1 (December 2008) DOI: 10.1016/j.jaci.2008.08.026 Copyright © 2008 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Clinical and histologic course of colitis after HSCT. A, Colonic biopsy specimens showing active colitis (before HSCT, 105 days after HSCT, and 20 months after HSCT), GVHD (105 days after HSCT; apoptosis is indicated by arrows in the inset), and resolution of both colitis and GVHD (30 months after HSCT). B, The dose of prednisolone equivalent is graphed as a function of time after HSCT with bacterial infections (arrows, B), relevant events, and treatments, as indicated. Journal of Allergy and Clinical Immunology 2008 122, 1113-1118.e1DOI: (10.1016/j.jaci.2008.08.026) Copyright © 2008 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 2 A-C, Cytokine production in response to TLR ligands. TNF-α production is normalized relative to adult control subjects assessed within each experiment. Data represent the mean plus SD of 2 to 3 independent assays performed at 4 to 8 months after HSCT. MALP, Macrophage-activating lipopeptide; Poly I:C, polyinosinic:polycytidylic acid; ODN2216, oligodeoxynucleotide 2216. Journal of Allergy and Clinical Immunology 2008 122, 1113-1118.e1DOI: (10.1016/j.jaci.2008.08.026) Copyright © 2008 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 3 Restoration of B-cell and NK cell function after HSCT. A, NK cell cytotoxicity. B, Proliferation of PBMCs to anti-CD40 plus IL-4 measured by means of incorporation of tritiated thymidine. Data are representative of 2 experiments performed at 8 to 9 months after HSCT. Journal of Allergy and Clinical Immunology 2008 122, 1113-1118.e1DOI: (10.1016/j.jaci.2008.08.026) Copyright © 2008 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Cytokine production in response to TLR4 agonist Cytokine production in response to TLR4 agonist. TNF production is normalized relative to adult control cells assessed within each experiment. Data represent mean plus SD of 2 independent assays performed at approximately 45 months after HSCT. Journal of Allergy and Clinical Immunology 2008 122, 1113-1118.e1DOI: (10.1016/j.jaci.2008.08.026) Copyright © 2008 American Academy of Allergy, Asthma & Immunology Terms and Conditions