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Kim E. Nichols, MD

Attending Physician

Director, Pediatric Hereditary Cancer Predisposition Program

Associate Professor of Pediatrics, Perelman School of Medicine at the University of Pennsylvania

Abstracts

 

2014

Cianferoni A, Barni S, Smith CL, Abram V, Bassiri H, Saretta F, Guan P, Ruyman K, Nichols KE, Spergel JM. Older mice intranasally sensitized with Aspergillus fumigatus develop stronger eosinophilic esophageal inflammation compared to their younger counterparts. AAAAI Annual Meeting, San Diego CA, February 2014.

2013

Spessott WA, Sanmillan ML, Nichols KE, Giraudo CG. R65Q mutation in STXBP2 sheds light on the molecular mechanisms that control lytic granule release [abstract]. Frontiers in Immunology; 2013.

Spessott WA, Sanmillan ML, Nichols KE, Giraudo CG. Novel mutation in STXBP2 sheds light on the molecular mechanisms that control lytic granule release [abstract]. 29th Annual Meeting of the Histiocyte Society; Washington DC, 2013 Oct.

Das R, Bassiri H, Guan P, Wiener SJ, Banerjee PP, Zhong M-C, et al. The adaptor molecule SAP plays essential roles during invariant NKT cell cytotoxicity and lytic synapse formation [abstract]. American Association of Immunologists; Honolulu HI, 2013 May.

Behrens EM, Nichols KE, Baratono S. Interferon gamma (IFNg) and Toll-like Receptor 9 (TLR9) provide collaborating and non-redundant signals to inhibit B-cell development in cytokine storm syndromes [abstract]. 29th Annual Meeting of the Histiocyte Society; Washington DC, 2013 Oct.

Guan P, Das R, Barrett DM, Maude SL, Teachey DT, Canna S, et al. Janus kinase inhibition as a novel treatment for the macrophage activation syndromes [abstract]. 29th Annual Meeting of the Histiocyte Society; Washington DC, 2013 Oct.

Teachey DT, Rheingold SR, Maude SL, Zugmaier G, Barrett DM, Seif AE, et al. Cytokine release syndrome after blinatumomab treatment related to abnormal macrophage activation and ameliorated with cytokine directed therapy [abstract]. 29th Annual Meeting of the Histiocyte Society; Washington DC, 2013 Oct.

2012

Das R, Bassiri H, Banergee P, Guan P, Wiener SJ, Orange JS, et al. Regulation of invariant natural killer T cell anti-tumor responses by the adaptor molecule signaling lymphocytic activation molecule-associated protein [abstract]. American Association of Immunologists; Boston MA, 2012 May.

Bassiri H, Das R, Guan P, Banerjee PP, Orange JS, Nichols KE. Invariant NKT cells mediate TCR- and perforin-dependent cytotoxicity against T cell lymphoma [abstract]. Clinical Immunology Society Annual Meeting: Primary Immunodeficiency Disease North American Conference; Vancouver BC, Canada, 2012 May.

Menard L, Tangye SG, Church J, Klion AD, Cunningham-Rundles C, Nichols KE, et al. SLAM-associated protein (SAP) is essential for the establishment of human B cell tolerance [abstract]. Clinical Immunology Society Annual Meeting: Primary Immunodeficiency Disease North American Conference; Vancouver BC, Canada, 2012 May.

Barr virus-induced hemophagocytic lymphohistiocytosis [abstract]. Pediatric Academic Society; Boston, MA, 2012 Apr.

Chellapandian D, Wiener S, Zelley K, Nichols KE. Treatment of EBV-HLH with rituximab containing regimens [abstract]. Eastern Society for Pediatric Research; Philadelphia, PA, 2012 Mar.

Wilkens A, Wertheim GBW, Fincher C, Bagatell R, Rheingold SR, Nichols KE, et al. Burkitt’s lymphoma in a child with mild Palliser-Killian Syndrome [abstract]. American College of Medical Genetics; Charlotte NC, 2012 Mar.

2011

Urtishak KA, Robinson BW, Biegel JA, Nichols KE, Felix CA. Unique familial MLL-rearranged precursor B cell infant acute lymphoblastic leukemia (ALL) in non-twin sibling [abstract]. American Society of Hematology; San Diego, CA, 2011 Dec.

Chellapandian D, Wiener S, Zelley K, Nichols KE. Safety and efficacy of rituximab in the treatment of Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis [abstract]. American Society of Hematology; San Diego, CA, 2011 Dec.

Weiner S, Zelley K, Nichols KE. Use of the anti-CD20 antibody rituximab in the treatment of Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis [abstract]. 27th Annual meeting of the Histiocyte Society; Vienna, Austria, 2011 Oct.

Das R, Bassiri H, Guan P, Nichols KE. Regulation of invariant natural killer T cell functions by the adaptor molecule signaling lymphocytic activation molecule-associated protein [abstract]. 6th Annual Symposium on CD1 and NKT cells; Chicago, IL, 2011 Sep.

Jyonouchi S, Abraham V, Orange JS, Spergel JS, Nichols KE, Cianferoni A. Invariant natural killer T cells from food allergic versus non-allergic children exhibit differential responsiveness to milk-derived sphingomyelin [abstract]. Keystone Symposia on Molecular and Cellular Biology; NK and NKT Cell Biology: Specificity and Redundancy of Innate Responses; Breckenridge, CO, 2011 Jan.

Bassiri H, Das R, Guan P, Nichols KE. Molecular requirements for NKT cell cytotoxic responses [abstract]. Keystone Symposia on Molecular and Cellular Biology; NK and NKT Cell Biology: Specificity and Redundancy of Innate Responses; Breckenridge, CO, 2011 Jan.

Das R, Bassiri H, Guan P, Nichols KE. Dichotomoous roles for SAP in mature NKT cell functions [abstract]. Keystone Symposia on Molecular and Cellular Biology; NK and NKT Cell Biology: Specificity and Redundancy of Innate Responses; Breckenridge, CO, 2011 Jan.

Awards

2013, Nezelof Prize for Basic Research, The Histiocyte Society
2013 Alex’s Lemonade Stand Foundation Innovation Award, Center for Childhood Cancer Research, Children’s Hospital of Philadelphia
2012 SAS Foundation for Cancer Research Award
2012 Alex’s Lemonade Stand Foundation Innovation Award, Center for Childhood Cancer Research, Children’s Hospital of Philadelphia
2011 Member, Society for Pediatric Research
2011 Center for Integration of Genetic Healthcare Technologies Award, University of Pennsylvania School of Medicine
2011 Vaccines & Immunologic Therapies Research Affinity Group Award, Children’s Hospital of Philadelphia
2011 Alex’s Lemonade Stand Foundation Seed Grant Award, Center for Childhood Cancer Research, Children’s Hospital of Philadelphia
2010, Foerderer Award, Children's Hospital of Philadelphia
2002, American Society of Hematology Junior Faculty Scholar Award 
2001, McCabe Foundation Award, University of Pennsylvania School of Medicine
2001, MAPS Award, Children's Hospital of Philadelphia
1999, ACS IRG, University of Pennsylvania School of Medicine
1999, Artemis Fellowship Award, Nikolas Symposium on the Histiocytosis
1998, Lee Fellowship Award, Dana-Farber Cancer Institute
1995, ACS Clinical Oncology Career Development Award (declined)
1995, Physician Scientist Award, National Institutes of Health
1989, Stead Research Fellowship, Duke University School of Medicine
1989, Alpha Omega Alpha, Duke University School of Medicine
1984, Summa Cum Laude, Dartmouth College
1984, Phi Beta Kappa, Dartmouth College
1980-84, Daniel Webster Scholar, Dartmouth College

Books

Chapters

2013

Hedayat M, Al-Herz W, Aghamohammadi A, Nichols KE, Rezaei N. Primary Immunodeficiencies and cancers. In Cancer Immunology: Translational Medicine from Bench to Bedside, Nima Rezaei (Ed). Springer Press, 2013.

Nichols KE, Marsh RM. The X-linked lymphoproliferative syndromes. In Immunologic Disorders in Infants and Children. 6th Edition. E. Richard Stiehm, Hans D. Ochs, Jerry A. Winkelstein, M.D (eds). Elsevier Saunders Press, 2013.

2012

Bassiri H, Nichols KE. X-linked lymphoproliferative syndrome. Atlas of Genetics and Cytogenetics in Oncology and Haematology; 2012 16(9): 685-688.

Knapke S, Zelley K, Nichols KE, Kohlman W, Schiffman J. Considerations for the identification, management and genetic evaluation of children with cancer predisposing conditions. American Society of Clinical Oncology Educational Program, 2012; 576-584.

Seif A, Biegel J, Lange BJ, Nichols KE. Inherited predisposition to childhood leukemia. In: Ching-Hon Pui, editor. Childhood leukemias. 3rd ed. New York: Cambridge University Press; 2012.

2011

Ganguly A, Nichols KE. The genetics of retinoblastoma: molecular and clinical aspects. In: Ramasubramanian A, Shields CL, editors. Retinoblastoma, 1st ed. New Delhi, India: Jaypee Brothers Medical Publishers; 2011.

2006

Nichols KE. Retinoblastoma. In: Leahey A, Meadows AT, editors. Requisites in pediatrics: hematology and oncology. Philadelphia: Elsevier Health Sciences; 2006.

Goldsmith K. Hogarty M, Nichols KE. The childhood histiocytoses: langerhans cell histiocytosis and hemophagocytic lymphohistiocytosis. In: Brodeur GM, Manno CS, editors. Requisites in pediatrics: hematology and oncology. Philadelphia: Elsevier Health Sciences; 2006.

2001

Nichols KE, Tangye S. The X-linked lymphoproliferative disease gene, SH2D1A. In: Encyclopedia of molecular medicine. New York: John Wiley & Sons, Inc.; 2001.

Tangye SG, Nichols, KE. Function of the T-cell co-stimulatory molecule, SLAM. In: Principles of molecular medicine, New York:John Wiley & Sons, Inc.; 2001.

1998

Nichols KE, Haber DA. Molecular genetics of renal neoplasms: Wilms' tumor and renal cell carcinoma. In: Jameson JL. Principles of molecular medicine. Humana Press; 1998. 

Editorial and Academic Positions

Editorial positions

Associate Editor, Frontiers in Primary Immunodeficiency
Member, editorial board, Clinical Immunology

Ad hoc reviewer

Blood
British Journal of Hematology
Clinical Cancer Research
Experimental Immunology
EMBO
European Journal of Hematology
European Journal of Immunology
Haematologica
Human Mutation
International Journal of Cancer
Journal of Clinical Investigation
Journal of Clinical Oncology
Journal of Experimental Medicine
Journal of Immunology
Journal of Leukocyte Biology
Journal of Medical Genetics
Journal of Orthopedic Research
Journal of Hematology/Oncology
Journal of Pediatric Ophthalmology & Strabismus
Journal of Pediatrics
Medical and Pediatric Oncology
Molecular and Cellular Biology
Molecular Medicine
Nature
Nature Reviews Immunology
New England Journal of Medicine
Pediatric Blood and Cancer
Pediatric Infectious Disease Journal
Proceedings of the National Academy of Science

Academic positions

2011 Member, Search Committee for investigator in stem cell  transplantation
2010-present Member, Search Committee for Investigator in Stem Cell transplantation, Children’s Hospital of Philadelphia
2009-present, Participant and Member, MD, PhD Admission Committee
2008-present Participant, Physician Scientist Recruitment and Retention Committee, Children’s Hospital of Philadelphia
2007-present Hematology-Oncology Fellowship Scientific Review Committee (ad hoc member), Children’s Hospital of Philadelphia

Leadership

Memberships in professional organizations

2013-present Member, HLH Steering Committee, Histiocyte Society
2012-present Member, Scientific Committee, Histiocyte Society
2012-present Member, Hematology Oncology Sub-board, American Board of Pediatrics
2011, American Society of Hematology

  • 2013, Member, Scientific Committee on Immunology and Host Defense

2010- present Member, Clinical Immunology Society
2009-present, Histiocyte Society, Macrophage Activation Subcommittee
2005-present, Histiocytosis Association of America
2004-present, Children's Oncology Group; Retinoblastoma Committee
1996-present, Histiocyte Society

New Media

Schneider K, Zelley K, Nichols KE, Garber J. Li-Fraumeni syndrome. GeneReviews at GeneTests: Medical Genetics Information Resource [database online]. Copyright, University of Washington, Seattle, 1997-2010; updated April 2013. Available at http://www.genetests.org.

Original Papers

2013

Bassiri H, Das R, Guan P, Barrett DM, Brennan PJ, Banerjee P, Wiener SJ, Orange JS, Brenner MB, Grupp SA, Nichols KE. iNKT cell cytotoxic responses control T-lymphoma growth in vitro and in vivo.Cancer Immunol Res. Published OnlineFirst October 21, 2013; doi:10.1158/2326-6066.CIR-13-0104. Read the paper

Chaigne-Delalande B, Li FY, O'Connor GM, Lukacs MJ, Jiang P, Zheng L, et al. Mg2+ regulates cytotoxic functions of NK and CD8 T cells in chronic EBV infection through NKG2D. Science. 2013 Jul 12;341(6142):186-91. doi: 10.1126/science.1240094. Cited in PubMed: PMID 23846901. Read the abstract

Kalish JM, Conlin LK, Bhatti TR, Dubbs HA, Harris MC, Izumi K, et al. Clinical features of three girls with mosaic genome-wide paternal uniparental isodisomy. Am J Med Genet A. 2013 Aug;161(8):1929-39. doi: 10.1002/ajmg.a.36045. Epub 2013 Jun 26. Cited in PubMed: PMID 23804593. Read the abstract

Teachey DT, Rheingold SR, Maude SL, Barrett DM, Seif AE, Nichols KE,et al. Cytokine release syndrome after blinatumomab treatment related to abnormal macrophage activation and ameliorated with cytokine directed therapy. Blood. 2013 Jun 27;121(26):5154-7. doi: 10.1182/blood-2013-02-485623. Epub 2013 May 15. Cited in PubMed: PMID 23678006. Read the abstract

Chellapandian D, Das R, Zelley K, Wiener S, Zhao H, Teachey DT, et al. Treatment of Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis with rituximab-containing immune-chemotherapeutic regimens. Br J Haematol. 2013 Aug;162(3):376-82. doi: 10.1111/bjh.12386. Epub 2013 May 21. Cited in PubMed: PMID 23692048. Read the abstract

May RM, Okumura M, Hsu C-J, Bassiri H, Rak G, Mace E, et al. Two distinct signaling pathways contribute to the function of NK cells stimulated through ITAM-bearing activating receptors. Blood. 2013 Apr 18;121(16):3135-46. doi: 10.1182/blood-2012-12-474361. Epub 2013 Feb 13. Cited in PubMed: PMID 23407547. Read the abstract

Kalish JM, Conlin LK, Mostoufi-Moab S, Wilkens AB, Mulchandani S, Zelley K, et al. Bilateral pheochromocytomas, hemihyperplasia, and subtle somatic mosaicism: the importance of detecting low-level uniparental disomy. Am J Med Genet A. 2013 May;161A(5):993-1001. doi: 10.1002/ajmg.a.35831. Epub 2013 Mar 26. Cited in PubMed: PMID 23532898. Read the abstract

Das R, Bassiri H, Guan P, Wiener S, Banerjee P, Zhong M-C, et al. The adaptor molecule SAP plays essential roles during invariant NKT cell cytotoxicity and lytic synapse formation. Blood. 2013 Apr 25;121(17):3386-95. doi: 10.1182/blood-2012-11-468868. Epub 2013 Feb 21. Cited in PubMed: PMID 23430111. Read the abstract

2012

Fiorino K, Lestini B, Nichols KE, Anupindi S, Maqbool A. An unusual case of gastrointestinal bleeding. Case Rep Pediatr. 2011;2011:748543. doi: 10.1155/2011/748543. Epub 2012 Jan 22. Cited in PubMed: PMID 22606522. Read the article

Palendira U, Low C, Bell AI, Ma CS, Abbott RJM, Phan TG, D et al. Expansion of somatically reverted memory CD8+ T cells in X-linked lymphoproliferative disease is due to selective pressure from Epstein Barr virus. J Exp Med. 2012 May 7;209(5):913-24. doi: 10.1084/jem.20112391. Epub 2012 Apr 9. Cited in PubMed: PMID 22493517. Read the article

Hassan A, Booth C, Brightwell A, Allwood Z, Veys P, Rao K, et al. A study of the inborn errors working party of the Eur. Group for Blood and Marrow Transplantation and Eur. Soc. for Immunodeficiency. Outcome of Haematopoietic Stem Cell Transplantation for Adenosine Deaminase Deficient Severe Combined Immunodeficiency. Blood. 2012 Oct 25;120(17):3615-24; quiz 3626. doi: 10.1182/blood-2011-12-396879. Epub 2012 Jul 12. Cited in PubMed: PMID 22791287. Read the abstract

2011

Booth C, Gilmour KC, Gennery AR, Slatter MA, Chapel H, Heath P, et al. X-linked Lymphoproliferative disease due to SAP/SH2D1A deficiency: a multicentre study on the manifestations, management and outcome of the disease. Blood. 2011 Jan 6;117(1):53-62. doi: 10.1182/blood-2010-06-284935. Epub 2010 Oct 6. Cited in PubMed: PMID 20926771. Read the article

Bunin GR, Felice MA, Davidson W, Friedman DL, Shields CL, Maidment A, et al. Medical radiation exposure and risk of retinoblastoma resulting from new germline RB1 mutation. Int J Cancer Suppl. 2011 May 15;128(10):2393-404. doi: 10.1002/ijc.25565. Cited in PubMed: PMCID: PMC3124307. Read the article

Nallasamy S, Eagle RC, Rorke-Adams, Nichols KE, Brucker AH. Eye findings in X-linked lymphoproliferative disease. Retina. 2011 Apr;31(4):790-7. doi: 10.1097/IAE.0b013e318206cd67. Cited in PubMed: PMID 21394060. Read the abstract

Jyonouchi S, Abraham V, Orange JS, Spergel JM, Gober L, Dudek E, et al. Invariant natural killer T cells from food allergic versus non-allergic children exhibit differential responsiveness to milk derived sphingomyelin. J Allergy Clin Immunol. 2011 Jul;128(1):102-109.e13. doi: 10.1016/j.jaci.2011.02.026. Epub 2011 Apr 1. Cited in PubMed: PMCID: PMC3129401.Read the article

Rothman JA, Das R, Teachey DT, Paessler ME, Nichols KE. Rapamycin does not control hemophagocytic lymphohistiocytosis in LCMV-infected perforin-deficient mice. Pediatr Blood Cancer. 2011 Dec 15;57(7):1239-43. doi: 10.1002/pbc.23226. Epub 2011 Jun 16. Cited in PubMed: PMID 21681935. Read the abstract

Rezaei N, Hedayat M, Aghamohammadi A, Nichols KE. Primary immunodeficiency diseases associated with increased susceptibility to viral infections and malignancies. J Allergy Clin Immunol. 2011 Jun;127(6):1329-41.e2;quiz 1342-3. Epub 2011 Apr 22. Cited in PubMed: PMID 21514636. Read the abstract

Baldanzi G, Pighini A, Bettio V, Rainero E, Traini S, Chianale F, et al. SAP-mediated inhibition of diacylglycerol kinase alpha regulates TCR-induced diacylglycerol signalling. J Immunol. 2011 Dec 1;187(11):5941-51. doi: 10.4049/jimmunol.1002476. Epub 2011 Nov 2. Cited in PubMed: 22048771. Read the article 

Teplick A, Kowalski M, Biegel JA, Nichols KE. Screening in cancer predisposition syndromes: guidelines for the general pediatrician. Eur J Pediatr. 2011 Mar;170(3):285-94. doi: 10.1007/s00431-010-1377-2. Epub 2011 Jan 6. Cited in PubMed: PMID 21210147. Read the article

Posters and Presentations

2013

Nichols K, JAnus kinase inhibition as a novel treatment for hemophagocytic lymphohistiocytosis, The 29th International meeting of the Histiocyte Society, Washington DC, October 2013

Alderfer M, Ford C, Zelley K, Lindell RB, Nichols KE. In their own words: Parents' decisions regarding testing children for LFS. Li Fraumeni Syndrome Conference, Boston MA, October 2013
Nichols K. Novel insights into anti-viral and anti-tumor immunity through the study of rare genetic disorders [oral presentation]. St. Jude Children’s Research Hospital, 2013 Jul 17; Memphis, TN.

Nichols K. Harnessing the anti-tumor functions of invariant NKT cells [oral presentation]. University of Pennsylvania Hematological Malignancies Research in Progress, 2013 Apr 25; Philadelphia, PA.

Nichols K. The missing link: genetic predisposition to childhood cancer [oral presentation]. CHOP Care Talks Cutting Edge Medicine in Every Day Practice, 2013 Jan 26; Plainsboro, NJ. 

2012

Nichols K. SAP signaling in macrophage activation syndrome [oral presentation]. American College of Rheumatology/Association of Rheumatology Health Professionals Annual Meeting, 2012 Nov 12; Washington, DC.

Nichols K. Molecular basis of familial HLH [oral presentation]. Thesis Opponent, Karolinska Institutet, 2012 Oct 12; Stockholm, Sweden.

Nichols K. The adaptor molecule SAP controls NKT cell cytotoxicity via effects on PKC-theta translocation to the immunologic synapse [oral presentation]. 2nd International XLP Symposium, 2012 Oct 8; London, England.

Nichols K. Diagnosis and treatment of the hemophagocytic lymphohistiocytoses [oral presentation]. Pediatric Intensive Care Unit, The Children’s Hospital of Philadelphia, 2012 Jun 28; Philadelphia, PA.

Nichols K. Molecular control of invariant natural killer T cell anti-tumor activity [oral presentation]. Normal & Malignant Hematopoiesis Research Affinity Group, University of Pennsylvania Hematologic Malignancies Program, 2012 Jun 25; Philadelphia, PA.

Nichols K. Issues to consider regarding cancer genetic testing of children [oral presentation]. American Society of Clinical Oncology Education Session, 2012 Jun 5; Chicago, IL.

Nichols K. Molecular control of Epstein-Barr virus infection [oral presentation]. Grand Rounds, Otolaryngology Program, University of Pennsylvania, School of Medicine, 2012 May 31; Philadelphia, PA.

Nichols K. Parent decision making and communication around TP53 genetic testing of children [oral presentation]. Center for Integration of Genomic and Healthcare Technologies, University of Pennsylvania, School of Medicine, 2012 May 9; Philadelphia, PA.

Nichols K. Pediatric cancer predisposition syndromes: recognition and management [oral presentation]. Tumor Board, Children’s Hospital of Philadelphia, 2012 Apr 26; Philadelphia, PA.

Nichols K. Harnessing the anti-tumor activities of invariant NKT cells [oral presentation]. Joint Retreat, Children’s Hospital of Philadelphia Research Institute, Normal & Malignant Hematopoiesis Research Affinity Group and Abramson Family Cancer Center of the University of Pennsylvania Hematologic Malignancies Program, 2012 Mar 9; Philadelphia, PA.

Nichols K. Signals controlling iNKT and PMN activation: adaptors at the front line of host defense [oral presentation]. Seminar, Immunology Research in Progress, University of Pennsylvania, 2012 Mar 9; Philadelphia, PA.

Nichols K. New insights into anti-EBV immunity and cancer through the study of X-linked lymphoproliferative disorder [oral presentation]. Allergy & Immunology Program, University of Pennsylvania, 2012 Jan 27; Philadelphia, PA. 

2011

Nichols K. Use of the anti-CD20 antibody Rituximab in the treatment of Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis [oral presentation]. Histiocyte Society, 27th Annual Meeting, Scientific Session, 2011 Oct 17; Vienna, Austria.

Nichols K. Regulation of invariant natural killer T cell functions by the adaptor molecule signaling lymphocytic activation molecule-associated protein [oral presentation]. 6th International Symposium on CD1 and NKT cells, 2011 Sep 23; Chicago, IL.

Nichols K. Conditional elimination of SAP reveals dichotomous roles in NKT cell activation [oral presentation]. 2011 Keystone Conference on NK and NKT cells, 2011 Jan 11; Breckenridge, CO.
 

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