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Rebecca Hatcher Buckley, MD

James Buren Sidbury Professor of Pediatrics, in the School of Medicine
Campus Mail: 127 MSRB1, Durham, NC 27710
Phone: (919) 684-3204
Email: 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

Selected Publications

Sarzotti-Kelsoe, M, Daniell, XG, Whitesides, JF, and Buckley, RH. "The long and the short of telomeres in bone marrow recipient SCID patients." Immunol Res 49, no. 1-3 (April 2011): 44-48.

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Buckley, RH. "Transplantation of hematopoietic stem cells in human severe combined immunodeficiency: longterm outcomes." Immunol Res 49, no. 1-3 (April 2011): 25-43. (Review)

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Buckley, RH. "B-cell function in severe combined immunodeficiency after stem cell or gene therapy: a review." J Allergy Clin Immunol 125, no. 4 (April 2010): 790-797. (Review)

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Railey, MD, Lokhnygina, Y, and Buckley, RH. "Long-term clinical outcome of patients with severe combined immunodeficiency who received related donor bone marrow transplants without pretransplant chemotherapy or post-transplant GVHD prophylaxis." J Pediatr 155, no. 6 (December 2009): 834-840.e1.

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Sarzotti-Kelsoe, M, Win, CM, Parrott, RE, Cooney, M, Moser, BK, Roberts, JL, Sempowski, GD, and Buckley, RH. "Thymic output, T-cell diversity, and T-cell function in long-term human SCID chimeras." Blood 114, no. 7 (August 13, 2009): 1445-1453.

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Aiuti, A, Cattaneo, F, Galimberti, S, Benninghoff, U, Cassani, B, Callegaro, L, Scaramuzza, S, Andolfi, G, Mirolo, M, Brigida, I, Tabucchi, A, Carlucci, F, Eibl, M, Aker, M, Slavin, S, Al-Mousa, H, Al Ghonaium, A, Ferster, A, Duppenthaler, A, Notarangelo, L, Wintergerst, U, Buckley, RH, Bregni, M, Marktel, S, Valsecchi, MG, Rossi, P, Ciceri, F, Miniero, R, Bordignon, C, and Roncarolo, M-G. "Gene therapy for immunodeficiency due to adenosine deaminase deficiency." N Engl J Med 360, no. 5 (January 29, 2009): 447-458.

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Griffith, LM, Cowan, MJ, Kohn, DB, Notarangelo, LD, Puck, JM, Schultz, KR, Buckley, RH, Eapen, M, Kamani, NR, O'Reilly, RJ, Parkman, R, Roifman, CM, Sullivan, KE, Filipovich, AH, Fleisher, TA, and Shearer, WT. "Allogeneic hematopoietic cell transplantation for primary immune deficiency diseases: current status and critical needs." J Allergy Clin Immunol 122, no. 6 (December 2008): 1087-1096.

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Boyle, JM, and Buckley, RH. "Population prevalence of diagnosed primary immunodeficiency diseases in the United States." J Clin Immunol 27, no. 5 (September 2007): 497-502.

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Palmer, K, Green, TD, Roberts, JL, Sajaroff, E, Cooney, M, Parrott, R, Chen, D-F, Reinsmoen, NL, and Buckley, RH. "Unusual clinical and immunologic manifestations of transplacentally acquired maternal T cells in severe combined immunodeficiency." J Allergy Clin Immunol 120, no. 2 (August 2007): 423-428.

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Roberts, JL, Lauritsen, JPH, Cooney, M, Parrott, RE, Sajaroff, EO, Win, CM, Keller, MD, Carpenter, JH, Carabana, J, Krangel, MS, Sarzotti, M, Zhong, X-P, Wiest, DL, and Buckley, RH. "T-B+NK+ severe combined immunodeficiency caused by complete deficiency of the CD3zeta subunit of the T-cell antigen receptor complex." Blood 109, no. 8 (April 15, 2007): 3198-3206.

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Atluri, S, Neville, K, Davis, M, Robertson, KA, Marshalleck, FE, O'Malley, DP, Buckley, RH, and Nelson, RP. "Epstein-Barr-associated leiomyomatosis and T-cell chimerism after haploidentical bone marrow transplantation for severe combined immunodeficiency disease." J Pediatr Hematol Oncol 29, no. 3 (March 2007): 166-172.

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Keller, MD, Chen, DF, Condron, SA, Liu, N, Reinsmoen, NL, and Buckley, RH. "The effect of natural killer cell killer Ig-like receptor alloreactivity on the outcome of bone marrow stem cell transplantation for severe combined immunodeficiency (SCID)." J Clin Immunol 27, no. 1 (January 2007): 109-116.

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Buckley, RH. "Primary immunodeficiency or not? Making the correct diagnosis." J Allergy Clin Immunol 117, no. 4 (April 2006): 756-758.

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Buckley, RH. "Variable phenotypic expression of mutations in genes of the immune system." J Clin Invest 115, no. 11 (November 2005): 2974-2976. (Review)

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Buckley, RH. "The multiple causes of human SCID." J Clin Invest 114, no. 10 (November 2004): 1409-1411.

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