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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

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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Farmer, JR, Ong, MS, Barmettler, S, Yonker, LM, Fuleihan, R, Sullivan, KE, Cunningham-Rundles, C, Walter, JE, Lugar, P, Suez, D, Routes, J, Bonilla, FA, Kleiner, G, Ballas, ZK, Secord, EA, Buckley, R, Joshi, A, Akhter, J, Puck, J, Haddad, E, Calabrese, L, Strober, W, Patel, NC, Ochs, HD, Uygungil, B, Stein, MR, Chen, K, Ballow, M, Bennett, N, Lehman, H, Dorsey, M, Fernandez, J, Caldwell, J, Hostoffer, R, Knight, A, Shapiro, R, Apter, AJ, Bennion, JR, Berger, M, Calderon, J, Cheng, L, and Cooper, M et al. "Common variable immunodeficiency non-infectious disease endotypes redefined using unbiased network clustering in large electronic datasets." Frontiers in Immunology 8, no. JAN (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, and Schulz, A et al. "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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Heimall, J, Logan, BR, Cowan, MJ, Notarangelo, LD, Griffith, LM, Puck, JM, Kohn, DB, Pulsipher, MA, Parikh, S, Martinez, C, Kapoor, N, O'Reilly, R, Boyer, M, Pai, S-Y, Goldman, F, Burroughs, L, Chandra, S, Kletzel, M, Thakar, M, Connelly, J, Cuvelier, G, Davila Saldana, BJ, Shereck, E, Knutsen, A, Sullivan, KE, DeSantes, K, Gillio, A, Haddad, E, Petrovic, A, Quigg, T, Smith, AR, Stenger, E, Yin, Z, Shearer, WT, Fleisher, T, Buckley, RH, and Dvorak, CC. "Immune reconstitution and survival of 100 SCID patients post-hematopoietic cell transplant: a PIDTC natural history study." Blood 130, no. 25 (December 2017): 2718-2727.

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Gernez, Y, Freeman, AF, Holland, SM, Garabedian, E, Patel, NC, Puck, JM, Sullivan, KE, Akhter, J, Secord, E, Chen, K, Buckley, R, Haddad, E, Ochs, HD, Fuleihan, R, Routes, J, Muskat, M, Lugar, P, Mancini, J, and Cunningham-Rundles, C. "Autosomal Dominant Hyper-IgE Syndrome in the USIDNET Registry." The journal of allergy and clinical immunology. In practice (September 19, 2017).

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Heimall, J, Buckley, RH, Puck, J, Fleisher, TA, Gennery, AR, Haddad, E, Neven, B, Slatter, M, Roderick, S, Baker, KS, Dietz, AC, Duncan, C, Griffith, LM, Notarangelo, L, Pulsipher, MA, and Cowan, MJ. "Recommendations for Screening and Management of Late Effects in Patients with Severe Combined Immunodeficiency after Allogenic Hematopoietic Cell Transplantation: A Consensus Statement from the Second Pediatric Blood and Marrow Transplant Consortium International Conference on Late Effects after Pediatric HCT." Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation 23, no. 8 (August 2017): 1229-1240. (Review)

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Heimall, J, Puck, J, Buckley, R, Fleisher, TA, Gennery, AR, Neven, B, Slatter, M, Haddad, E, Notarangelo, LD, Baker, KS, Dietz, AC, Duncan, C, Pulsipher, MA, and Cowan, MJ. "Current Knowledge and Priorities for Future Research in Late Effects after Hematopoietic Stem Cell Transplantation (HCT) for Severe Combined Immunodeficiency Patients: A Consensus Statement from the Second Pediatric Blood and Marrow Transplant Consortium International Conference on Late Effects after Pediatric HCT." Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation 23, no. 3 (March 2017): 379-387. (Review)

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Wu, EY, Ehrlich, L, Handly, B, Frush, DP, and Buckley, RH. "Clinical and imaging considerations in primary immunodeficiency disorders: an update." Pediatric radiology 46, no. 12 (November 2016): 1630-1644. (Review)

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Sayour, EJ, Mousallem, T, Van Mater, D, Wang, E, Martin, P, Buckley, RH, and Barfield, RC. "Bone marrow transplantation for CVID-like humoral immune deficiency associated with red cell aplasia." Pediatric blood & cancer 63, no. 10 (October 2016): 1856-1859.

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