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Rebecca Hatcher Buckley, MD

James Buren Sidbury Professor of Pediatrics, in the School of Medicine
Professor of Pediatrics
Professor of Immunology in the Department of Immunology
Member of the Duke Cancer Institute
Campus mail: 127 MSRB1, Durham, NC 27710
Phone: (919) 684-3204
Email address: buckl003@mc.duke.edu

The overall emphasis of Dr. Buckley's research is in human T,B and NK cell development and in aberrations in their development and regulation. The work involves three particular areas of investigation: 1) the cellular and molecular bases of genetically-determined human immunodeficiency diseases, 2) the use of bone marrow stem cells to cure genetically-determined immunodeficiency diseases, and 3) the use of human SCID bone marrow stem cell chimeras to study human thymic education, T and B cell ontogeny, tolerance induction and MHC restriction mechanisms. Methodology includes monoclonal antibody (mAb) analyses of lymphocyte phenotypes, a variety of T cell and natural killer (NK) cell functional assays, studies of thymic output by T cell receptor recombination excision circle measurement, studies of T cell diversity by spectratyping, studies of T cell longevity by telomere analysis and assessment of B cell differentiation and function. A unique resource available for her studies is the largest populations of patients with genetically-determined immunodeficiency diseases in the U.S., which includes the largest population in the world of longterm SCID chimeras treated at a single center, some of whom have been studied and followed for more than 33 years. The administration of rigorously T cell depleted haploidentical bone marrow stem cells to SCID recipients without pre-transplant conditioning or post-transplant use of immunosuppressive drugs to prevent GVHD provides an unmanipulated system for studying human thymic education, T and B cell ontogeny, MHC restriction mechanisms and tolerance induction. Studies to identify mutations in patients with primary immunodeficiency are continuing, particularly in those with SCID.

Education and Training

  • M.D., University of North Carolina at Chapel Hill, 1958

Selected Grants and Awards

Publications

Haddad, E, Logan, BR, Griffith, LM, Buckley, RH, Parrott, RE, Prockop, SE, Small, TN, Chaisson, J, Dvorak, CC, Murnane, M, Kapoor, N, Abdel-Azim, H, Hanson, IC, Martinez, C, Bleesing, JJH, Chandra, S, Smith, AR, Cavanaugh, ME, Jyonouchi, S, Sullivan, KE, Burroughs, L, Skoda-Smith, S, Haight, AE, Tumlin, AG, Quigg, TC, Taylor, C, Dávila Saldaña, BJ, Keller, MD, Seroogy, CM, Desantes, KB, Petrovic, A, Leiding, JW, Shyr, DC, Decaluwe, H, Teira, P, Gillio, AP, Knutsen, AP, Moore, TB, Kletzel, M, Craddock, JA, Aquino, V, Davis, JH, Yu, LC, Cuvelier, GDE, Bednarski, JJ, Goldman, FD, Kang, EM, Shereck, E, Porteus, MH, Connelly, JA, Fleisher, TA, Malech, HL, Shearer, WT, Szabolcs, P, Thakar, MS, Vander Lugt, MT, Heimall, J, Yin, Z, Pulsipher, MA, Pai, S-Y, Kohn, DB, Puck, JM, Cowan, MJ, O'Reilly, RJ, and Notarangelo, LD. "SCID genotype and 6-month posttransplant CD4 count predict survival and immune recovery." Blood 132, no. 17 (October 2018): 1737-1749.

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Dvorak, CC, Haddad, E, Buckley, RH, Cowan, MJ, Logan, B, Griffith, LM, Kohn, DB, Pai, S-Y, Notarangelo, L, Shearer, W, Prockop, S, Kapoor, N, Heimall, J, Chaudhury, S, Shyr, D, Chandra, S, Cuvelier, G, Moore, T, Shenoy, S, Goldman, F, Smith, AR, Sunkersett, G, Vander Lugt, M, Caywood, E, Quigg, T, Torgerson, T, Chandrakasan, S, Craddock, J, Dávila Saldaña, BJ, Gillio, A, Shereck, E, Aquino, V, DeSantes, K, Knutsen, A, Thakar, M, Yu, L, and Puck, JM. "The genetic landscape of severe combined immunodeficiency in the United States and Canada in the current era (2010-2018)." The Journal of Allergy and Clinical Immunology (September 5, 2018).

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Belderbos, ME, Gennery, AR, Dvorak, CC, Blok, H-J, Eikema, D-J, Silva, JMF, Veys, P, Neven, B, Buckley, R, Cole, T, Cowan, MJ, Goebel, WS, Hoenig, M, Kuo, CY, Stiehm, ER, Wynn, R, and Bierings, M. "Outcome of domino hematopoietic stem cell transplantation in humans – an international case series." Journal of Allergy and Clinical Immunology (July 2018).

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Miggelbrink, AM, Logan, BR, Buckley, RH, Parrott, RE, Dvorak, CC, Kapoor, N, Abdel-Azim, H, Prockop, SE, Shyr, D, Decaluwe, H, Hanson, IC, Gillio, A, Dávila Saldaña, BJ, Eibel, H, Hopkins, G, Walter, JE, Whangbo, JS, Kohn, DB, Puck, JM, Cowan, MJ, Griffith, LM, Haddad, E, O'Reilly, RJ, Notarangelo, LD, and Pai, S-Y. "B-cell differentiation and IL-21 response in IL2RG/JAK3 SCID patients after hematopoietic stem cell transplantation." Blood 131, no. 26 (June 2018): 2967-2977.

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Giannelou, A, Wang, H, Zhou, Q, Park, YH, Abu-Asab, MS, Ylaya, K, Stone, DL, Sediva, A, Sleiman, R, Sramkova, L, Bhatla, D, Serti, E, Tsai, WL, Yang, D, Bishop, K, Carrington, B, Pei, W, Deuitch, N, Brooks, S, Edwan, JH, Joshi, S, Prader, S, Kaiser, D, Owen, WC, Sonbul, AA, Zhang, Y, Niemela, JE, Burgess, SM, Boehm, M, Rehermann, B, Chae, J, Quezado, MM, Ombrello, AK, Buckley, RH, Grom, AA, Remmers, EF, Pachlopnik, JM, Su, HC, Gutierrez-Cruz, G, Hewitt, SM, Sood, R, Risma, K, and Calvo, KR et al. "Aberrant tRNA processing causes an autoinflammatory syndrome responsive to TNF inhibitors." Annals of the rheumatic diseases 77, no. 4 (April 2018): 612-619.

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Mayor, PC, Eng, KH, Singel, KL, Abrams, SI, Odunsi, K, Moysich, KB, Fuleihan, R, Garabedian, E, Lugar, P, Ochs, HD, Bonilla, FA, Buckley, RH, Sullivan, KE, Ballas, ZK, Cunningham-Rundles, C, and Segal, BH. "Cancer in primary immunodeficiency diseases: Cancer incidence in the United States Immune Deficiency Network Registry." The Journal of allergy and clinical immunology 141, no. 3 (March 2018): 1028-1035.

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Baloh, CH, Reddy, A, Buckley, RH, and Lugar, PL. "Characterization of pediatric onset common variable immunodeficiency (CVID) in a large cohort." American-Academy-of-Allergy-Asthma-and-Immunology / World-Allergy-Organization Joint Congress. Orlando, FL. March 2, 2018 - March 8, 2018.: MOSBY-ELSEVIER, February 1, 2018.

Scholars@Duke

Baloh, CH, Reddy, A, Buckley, RH, and Lugar, PL. "Characterization of pediatric onset common variable immunodeficiency (CVID) in a large cohort." American-Academy-of-Allergy-Asthma-and-Immunology / World-Allergy-Organization Joint Congress. Orlando, FL. March 2, 2018 - March 8, 2018.: MOSBY-ELSEVIER, February 1, 2018.

Scholars@Duke

Farmer, JR, Ong, M-S, Barmettler, S, Yonker, LM, Fuleihan, R, Sullivan, KE, Cunningham-Rundles, C, and Walter, JE. "Common Variable Immunodeficiency Non-Infectious Disease Endotypes Redefined Using Unbiased Network Clustering in Large Electronic Datasets." Frontiers in Immunology 8 (January 9, 2018).

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Slack, J, Albert, MH, Balashov, D, Belohradsky, BH, Bertaina, A, Bleesing, J, Booth, C, Buechner, J, Buckley, RH, Ouachée-Chardin, M, Deripapa, E, Drabko, K, Eapen, M, Feuchtinger, T, Finocchi, A, Gaspar, HB, Ghosh, S, Gillio, A, Gonzalez-Granado, LI, Grunebaum, E, Güngör, T, Heilmann, C, Helminen, M, Higuchi, K, Imai, K, Kalwak, K, Kanazawa, N, Karasu, G, Kucuk, ZY, Laberko, A, Lange, A, Mahlaoui, N, Meisel, R, Moshous, D, Muramatsu, H, Parikh, S, Pasic, S, Schmid, I, Schuetz, C, Schulz, A, Schultz, KR, Shaw, PJ, Slatter, MA, Sykora, K-W, Tamura, S, Taskinen, M, Wawer, A, Wolska-Kuśnierz, B, Cowan, MJ, Fischer, A, Gennery, AR, Inborn Errors Working Party of the European Society for Blood and Marrow Transplantation and the European Society for Immunodeficiencies, , Stem Cell Transplant for Immunodeficiencies in Europe (SCETIDE), , Center for International Blood and Marrow Transplant Research, , and Primary Immunodeficiency Treatment Consortium, . "Outcome of hematopoietic cell transplantation for DNA double-strand break repair disorders." The Journal of Allergy and Clinical Immunology 141, no. 1 (January 2018): 322-328.e10.

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