Purine metabolism, immune reconstitution, and abdominal adipose tumor after gene therapy for adenosine deaminase deficiency Eyal Grunebaum, MD, Catherine.

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Presentation transcript:

Purine metabolism, immune reconstitution, and abdominal adipose tumor after gene therapy for adenosine deaminase deficiency Eyal Grunebaum, MD, Catherine T.-S. Chung, MD, Harjit Dadi, PhD, Peter Kim, MD, PhD, Immacolata Brigida, BSc, Francesca Ferrua, MD, Maria P. Cicalese, MD, Alessandro Aiuti, MD, Chaim M. Roifman, MD Journal of Allergy and Clinical Immunology Volume 127, Issue 6, Pages 1417-1419.e3 (June 2011) DOI: 10.1016/j.jaci.2011.04.014 Copyright © 2011 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Analysis of the abdominal tumor. Magnetic resonance imaging (upper left panel) demonstrated a large right-sided abdominal tumor (arrow) near the liver (arrowhead). The tumor had a diffuse, homogenous high signal on T1 and T2 sequences isointense to the subcutaneous fat, with no evidence of cystic changes or calcification. Gross inspection of the tumor revealed a well-circumscribed, thinly encapsulated tumor confined to the mesentery (upper right panel). Hematoxylin and eosin staining of the resected tumor demonstrated mature adipocytes with no cytologic atypia or mitoses (lower left panel). G-banding analysis of the resected tumor showed balanced inverted insertion of 8q11.23-q24 into chromosome 19 at 19q13.13, which is typical of lipoblastoma (lower right panel). Journal of Allergy and Clinical Immunology 2011 127, 1417-1419.e3DOI: (10.1016/j.jaci.2011.04.014) Copyright © 2011 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig E1 T-cell receptor diversity. Relative expression of the various Vβ families among CD4+ (upper panel) and CD8+ (lower panel) T cells in the patient 3 years after gene therapy (solid bars) or normal age-matched control subjects (open bars ± SD), as determined by means of fluorescence-activated cell sorting analysis with the IOTest Beta Mark Kit (Beckman Coulter, Mississauga, Ontario, Canada). Journal of Allergy and Clinical Immunology 2011 127, 1417-1419.e3DOI: (10.1016/j.jaci.2011.04.014) Copyright © 2011 American Academy of Allergy, Asthma & Immunology Terms and Conditions