by M. Louise Markert, Marilyn J

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Postnatal thymus transplantation with immunosuppression as treatment for DiGeorge syndrome by M. Louise Markert, Marilyn J. Alexieff, Jie Li, Marcella Sarzotti, Daniel A. Ozaki, Blythe H. Devlin, Debra A. Sedlak, Gregory D. Sempowski, Laura P. Hale, Henry E. Rice, Samuel M. Mahaffey, and Michael A. Skinner Blood Volume 104(8):2574-2581 October 15, 2004 ©2004 by American Society of Hematology

Survival of patients with complete DiGeorge syndrome treated with Thymoglobulin prior to thymus transplantation. . Survival of patients with complete DiGeorge syndrome treated with Thymoglobulin prior to thymus transplantation. M. Louise Markert et al. Blood 2004;104:2574-2581 ©2004 by American Society of Hematology

Immune function. Immune function. After transplantation, there is a variable change in (A) CD3, (B) CD4, and (C) CD8 T-cell numbers as clonal T-cell populations decrease in number. After transplantation there are increasing numbers of (D) naive CD4 T cells and (E) naive CD8 T cells and increasing (F) T-cell proliferative responses to PHA. Changes in the (G) absolute eosinophil counts and (H) TREC values are shown. For panels A to C, the 25th percentile for children ages one to 6 years22 is indicated by the dotted line. For panels D to E, the lowest value for 11 children aged one year23 is indicated by the dotted line; the lowest value for 9 adults23 is indicated by the solid line. In panel F, the mean for healthy adults is indicated by the solid line and the range of 2 standard deviations by the dotted lines. The patients' PBMC background proliferations (cells plus medium without mitogen) for the PHA assays (n=80) had a mean of 198 cpm with one standard deviation ranging from 74 cpm to 527 cpm. The normal control background had a mean of 173 cpm with one standard deviation ranging from 61 to 486 cpm. M. Louise Markert et al. Blood 2004;104:2574-2581 ©2004 by American Society of Hematology

Immunoscope evaluation. Immunoscope evaluation. Evaluation of DIG102, DIG106, and DIG107 before and after thymus transplantation. The RT-PCR was done on CD4 RNA for the posttransplantation panels of DIG106 and DIG107; it was done on PBMC RNA for the other panels. The final panel includes the identification of the TCRBV families. Examples of oligoclonal (O), polyclonal skewed (S), and polyclonal Gaussian-like (G) panels are indicated in the DIG102 profiles. The pretransplantation profiles have been reported17 and are reprinted with permission from the American Academy of Allergy, Asthma, and Immunology. Copyright 2004. M. Louise Markert et al. Blood 2004;104:2574-2581 ©2004 by American Society of Hematology

Thymus histology following transplantation. Thymus histology following transplantation. (A,C) Normal thymus; (B,D) biopsy from DIG107 conducted at 3 months after thymus transplantation. Panels A and B show staining with CD3 antibody (original magnification ×10). Panels C and D show staining with cytokeratin. Positive reaction with antibody is indicated by the brown color. Both cortex (C) and medulla (M) containing light lacy cytokeratin-positive epithelial cells and Hassall bodies are evident in the transplant biopsy from this patient. M. Louise Markert et al. Blood 2004;104:2574-2581 ©2004 by American Society of Hematology