Rebecca H. Buckley, MD

Department / Division:
Pediatrics / Allergy and Immunology

Address:
DUMC 2898
Durham, NC 27710

Appointment Telephone:
919-684-2922

Office Telephone:
919-684-2922

Fax Telephone:
919-681-7979

• MD, University of North Carolina at Chapel Hill School of Medicine, 1958

• Pediatrics, Duke University Medical Center, 1958-1961

• Pediatric Allergy, Duke University Medical Center, 1961-1963
• Immunology, Duke University Medical Center, 1963-1965

Clinical Interests:
All allergic diseases (e.g., asthma, allergic rhinitis, atopic dermatitis, urticaria, anaphylaxis), recurrent infections, primary immunodeficiency (e.g., IgA deficiency, agammaglobulinemia, chronic granulomatous disease, severe combined immunodeficiency or SCID), and bone marrow transplantation for SCID

Research Interests:
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, 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. 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.

Representative Publications:
Sarzotti M, Patel DD, Li X, Ozaki DA, Cao S, Langdon S, Parrott RE, Coyne K, Buckley RH. T Cell Repertoire Development in Humans with SCID After Nonablative Allogeneic Marrow Transplantation. J Immunol. 2003 Mar 1;170(5):2711-8. (2003) Abstract

Buckley RH. Treatment options for genetically determined immunodeficiency. Lancet. 2003 Feb 15;361(9357):541-2. (2003) Abstract

Buckley RH. 27. Transplantation immunology: Organ and bone marrow. J Allergy Clin Immunol. 2003 Feb;111(2 Suppl):733-44. (2003) Abstract

Myers LA, Patel DD, Puck JM, Buckley RH. Hematopoietic stem cell transplantation for severe combined immunodeficiency in the neonatal period leads to superior thymic output and improved survival. Blood. 2002 Feb 1;99(3):872-8. (2002) Abstract

Buckley RH. Primary immunodeficiency diseases: dissectors of the immune system. Immunol Rev. 2002 Jul;185(1):206-19. (2002) Abstract

Buckley RH. Primary cellular immunodeficiencies. J Allergy Clin Immunol. 2002 May;109(5):747-57. (2002) Abstract

Buckley RH. Immunoglobulin G subclass deficiency: fact or fancy?  Curr Allergy Asthma Rep.  2002 Sep;2(5):356-60. (2002) Abstract

Buckley RH. Gene therapy for SCID--a complication after remarkable success. Lancet 360: 1185-1186, 2002. (2002)

Buckley RH. American Pediatric Society Presidential Address 2000: reflections on the 20th and 21st centuries. Pediatr Res. 2002 Jan;51(1):119-23. (2002) Abstract

Morra M, Silander O, Calpe-Flores S, Choi M, Oettgen H, Myers L, Etzioni A, Buckley RH, and Terhorst C: Alterations of the X-linked lymphoproliferative disease gene SH2D1A  in the Common Variable Immunodeficiency syndrome. Blood 98: 1321-1325, 2001. (2001)

Buckley RH:  Bone marrow reconstitution in primary immunodeficiency.  Chapter 101 in Rich et al (ed): Clinical Immunology: Principles and Practice, Edition  II, Mosby-Yearbook, Inc., St. Louis, MO, 101.1-101.6, 2001 (2001)

Buckley RH: Primary immunodeficiencies of lymphocytes. Chapter 28 in Samter's Immunological Diseases, 6th Edition, 317-329, 2001. (2001)

Zhou YJ, Chen M, Cusack NA, Kimmel LH, Magnuson KS, Boyd JG, Lin W, Roberts JL, Lengi A, Buckley RH, Geahlen RL, Candotti F, Gadina M, Changelian PS, O'Shea JJ. Unexpected effects of FERM domain mutations on catalytic activity of Jak3: structural implication for Janus kinases. Mol Cell. 2001 Nov;8(5):959-69. (2001) Abstract

Yin EZ, Frush DP, Donnelly LF, Buckley RH. Primary immunodeficiency disorders in pediatric patients: clinical features and imaging findings. AJR Am J Roentgenol. 2001 Jun;176(6):1541-52. (2001) Abstract

Buckley RH. The hyper-IgE syndrome. Clin Rev Allergy Immunol. 2001 Feb;20(1):139-54. (2001) Abstract

Antaya RJ, Gardner ES, Bettencourt MS, Daines M, Denise Y, Uthaisangsook S, Buckley RH, Prose NS. Cutaneous complications of BCG vaccination in infants with immune disorders: two cases and a review of the literature.  Pediatr Dermatol.  ;18(3):205-9, 2001. (2001) Abstract

Winkelstein JA, Marino MC, Johnston RB Jr, Boyle J, Curnutte J, Gallin JI, Malech HL, Holland SM, Ochs H, Quie P, Buckley RH, Foster CB, Chanock SJ, Dickler H. Chronic granulomatous disease. Report on a national registry of 368 patients. Medicine (Baltimore). 2000 May;79(3):155-69. (2000) Abstract

Patel DD, Gooding ME, Parrott RE, Curtis KM, Haynes BF, Buckley RH. Thymic function after hematopoietic stem-cell transplantation for the treatment of severe combined immunodeficiency. N Engl J Med. 2000 May 4;342(18):1325-32. (2000) Abstract

Minegishi Y, Lavoie A, Cunningham-Rundles C, Bédard PM, Hébert J, Côté L, Dan K, Sedlak D, Buckley RH, Fischer A, Durandy A, Conley ME. Mutations in activation-induced cytidine deaminase in patients with hyper IgM syndrome. Clin Immunol. 2000 Dec;97(3):203-10. (2000) Abstract

Buckley RH. Primary immunodeficiency diseases due to defects in lymphocytes. N Engl J Med. 2000 Nov 2;343(18):1313-24. (2000) Abstract

Buckley RH. Gene therapy for human SCID: dreams become reality. Nat Med. 2000 Jun;6(6):623-4. (2000) Abstract

Buckley RH. Advances in the understanding and treatment of human severe combined immunodeficiency. Immunol Res. 2000;22(2-3):237-51. (2000) Abstract

Markert ML, Boeck A, Hale LP, Kloster AL, McLaughlin TM, Batchvarova MN, Douek DC, Koup RA, Kostyu DD, Ward FE, Rice HE, Mahaffey SM, Schiff SE, Buckley RH, Haynes BF. Transplantation of thymus tissue in complete DiGeorge syndrome. N Engl J Med. 1999 Oct 14;341(16):1180-9. (1999) Abstract

Grimbacher B, Schäffer AA, Holland SM, Davis J, Gallin JI, Malech HL, Atkinson TP, Belohradsky BH, Buckley RH, Cossu F, Español T, Garty BZ, Matamoros N, Myers LA, Nelson RP, Ochs HD, Renner ED, Wellinghausen N, Puck JM. Genetic linkage of hyper-IgE syndrome to chromosome 4. Am J Hum Genet. 1999 Sep;65(3):735-44. (1999) Abstract

Buckley RH: Primary Immunodeficiency Diseases. In Bennett JC, Plum F (eds): Cecil Textbook of Medicine, 21st ed, Philadelphia PA, W.B. Saunders Co, 1433-1440, 1999. (1999)

Buckley RH: Cellular therapy for severe combined immunodeficiency: transplant options and impact of donor type and graft manipulation on long-term graft function and completeness of immune reconstitution. Cancer Research, Therapy and Control 9:73-79, 1999 (1999)

Buckley RH, Schiff SE, Schiff RI, Markert L, Williams LW, Roberts JL, Myers LA, Ward FE. Hematopoietic stem-cell transplantation for the treatment of severe combined immunodeficiency. N Engl J Med. 1999 Feb 18;340(7):508-16. (1999) Abstract

Buckley RH, Myers LA: Treatment of severe combined immunodeficiency (Letter). N Engl J Med 341:291-292, 1999. (1999)

Buckley RH, Fischer A: Bone Marrow Transplantation for Primary Immunodeficiency Diseases. Chapter 37 in Ochs HD, Puck J, Smith CE (eds): Primary Immunodeficiency Diseases: A Molecular and Genetic Approach, Oxford Universitiy Press, New York, 1999, pgs 459-475. (1999)

Puel A, Ziegler SF, Buckley RH, Leonard WJ. Defective IL7R expression in T(-)B(+)NK(+) severe combined immunodeficiency. Nat Genet. 1998 Dec;20(4):394-7. (1998) Abstract

Markert ML, Hummell DS, Rosenblatt HM, Schiff SE, Harville TO, Williams LW, Schiff RI, Buckley RH. Complete DiGeorge syndrome: persistence of profound immunodeficiency. J Pediatr. 1998 Jan;132(1):15-21. (1998) Abstract

Laughlin MJ, Rizzieri DA, Smith CA, Moore JO, Lilly S, McGaughey D, Martin P, Carrier C, Stevens CE, Rubinstein P, Buckley RH, Kurtzberg J: Hematologic engraftment and reconstitution of immune function post unrelated placental cord blood transplant in an adult with acute lymphocytic leukemia. Leukemia Research 22:215-219, 1998. (1998)

Jabara HH, Buckley RH, Roberts JL, Lefranc G, Loiselet J, Khalil G, Geha RS. Role of JAK3 in CD40-mediated signaling. Blood. 1998 Oct 1;92(7):2435-40. (1998) Abstract

Buckley RH: Primary Immunodeficiency Diseases. In Paul WE (ed): Fundamental Immunology. Lippencott-Raven Press, Philadelphia, 4th edition, pp 1437-1463, 1998. Buckley RH: Commentary: Agammaglobulinemia, by Col. Ogden Bruton, MC, USA, Pediatrics 1952. Pediatrics 102:213-215, 1998. (1998)

Buckley RH: Primary Immunodeficiency Diseases. In Middleton E Jr, Reed CE, Ellis EF, Adkinson NF Jr, Yunginger JW (eds): Allergy, Principles and Practice. C.V. Mosby, St. Louis, 5th edition, pp 713-734, 1998. (1998)

Puck JM, Pepper AE, Henthorn PS, Candotti F, Isakov J, Whitwam T, Conley ME, Fischer RE, Rosenblatt HM, Small TN, Buckley RH. Mutation analysis of IL2RG in human X-linked severe combined immunodeficiency. Blood. 1997 Mar 15;89(6):1968-77. (1997) Abstract

Parker W, Yu PB, Holzknecht ZE, Lundberg K, Buckley RH, Platt JL. Specificity and function of "natural" antibodies in immunodeficient subjects: clues to B cell lineage and development. J Clin Immunol. 1997 Jul;17(4):311-21. (1997) Abstract

Markert ML, Kostyu DD, Ward FE, McLaughlin TM, Watson TJ, Buckley RH, Schiff SE, Ungerleider RM, Gaynor JW, Oldham KT, Mahaffey SM, Ballow M, Driscoll DA, Hale LP, Haynes BF. Successful formation of a chimeric human thymus allograft following transplantation of cultured postnatal human thymus. J Immunol. 1997 Jan 15;158(2):998-1005. (1997) Abstract

Davis CM, McLaughlin TM, Watson TJ, Buckley RH, Schiff SE, Hale LP, Haynes BF, Markert ML. Normalization of the peripheral blood T cell receptor V beta repertoire after cultured postnatal human thymic transplantation in DiGeorge syndrome. J Clin Immunol. 1997 Mar;17(2):167-75. (1997) Abstract

Buckley RH, Schiff RI, Schiff SE, Markert ML, Williams LW, Harville TO, Roberts JL, Puck JM. Human severe combined immunodeficiency: genetic, phenotypic, and functional diversity in one hundred eight infants. J Pediatr. 1997 Mar;130(3):378-87. (1997) Abstract