X. Long Zheng, MD, PhD

Appointments and Referrals: 1-800-TRY-CHOP (1-800-879-2467)

  • Background

    Clinical Expertise

    • Clinical pathology
    • Thrombosis and hemostasis
    • Transfusion medicine, particularly thrombotic micoangiopathies

    Lab Expertise

    • Thrombosis
    • Hemostasis
    • Coagulation
    • Platelet biology

    Research Interests

    • Thrombotic microangiopathies (TTP and HUS)
    • Hemophilia A
    • Platelet defects
    • Von Willebrand Disease
  • Education and Training

    Medical School

    Jiangxi Medical College, China


    Clinical Pathology, Barnes-Jewish Hospital, Washington University School of Medicine, St Louis, Mo.


    Transfusion Medicine, Barnes-Jewish Hospital, Washington University School of Medicine, St Louis, Mo.

    Board Certification

    American Board of Pathology/Blood Banking and Transfusion
    American Board of Pathology/Clinical Pathology

    Graduate Degree

    PhD, Molecular and Cell Biology, University of Vienna School of Medicine, Vienna, Austria

  • Titles and Academic Titles

    Medical Director, Coagulation Laboratory

    Medical Director, Hematology Laboratory

    Assistant Professor of Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania

  • Departments and Services
  • Publications



    Jobes D, Wolfe Y, O'Neill D, Calder J, Jones L, Sesok-Pizzini D, Zheng XL. Toward a definition of "fresh" whole blood: an in vitro characterization of coagulation properties in refrigerated whole blood for transfusion. Transfusion. 2010 Jul 15. [Epub ahead of print]. Read abstract

    Jin SY, Skipwith CG, Zheng XL. Amino acid residues Arg(659), Arg(660), and Tyr(661) in the spacer domain of ADAMTS13 are critical for cleavage of von Willebrand factor. Blood. 2010 Mar 18;115(11):2300-10. Epub 2010 Jan 14.Read abstract

    Zheng XL, Wu HM, Shang D, Falls E, Skipwith CG, Cataland SR, Bennett CL, Kwaan HC. Multiple domains of ADAMTS13 are targeted by autoantibodies against ADAMTS13 in patients with acquired idiopathic thrombotic thrombocytopenic purpura. Haematologica. 2010 Apr 7. [Epub ahead of print]. Read abstract


    Zheng XL, Kitamoto Y, Sadler JE. Enteropeptidase, a type II transmembrane serine protease. Front Biosci (Elite Ed). 2009 Jun 1;1:242-9. Review. Read abstract

    Skipwith C, Cao W, Zheng XL. Factor VIII and platelets synergistically accelerate cleavage of von Willebrand factor by ADAMTS13 under fluid shear stress. J Biol Chem. 2010 Jul 6. Read abstract.


    Zakarija A, Kwaan HC, Moake JL, Bandarenko N, Pandey DK, McKoy JM, Yarnold PR, Raisch DW, Winters JL, Raife TJ, Cursio JF, Luu TH, Richey EA, Fisher MJ, Ortel TL, Tallman MS, Zheng XL, Matsumoto M, Fujimura Y, Bennett CL. Ticlopidine- and clopidogrel-associated thrombotic thrombocytopenic purpura (TTP): review of clinical, laboratory, epidemiological, and pharmacovigilance findings (1989-2008). Kidney Int (Suppl) 112: S20-4.

    Zheng XL, Kitamoto Y,  Sadler JE. Enteropeptidase, a type II transmembrane serine protease. Frontiers Bioscience. E1: 606-613

    Laje P, Shang D, Cao W, Niiya M, Endo M, Radu A, DeRagotis N, Scheiflinger F, Zoltick PW, Flake AW, Zheng XL. Correction of Murine ADAMTS13 Deficiency by Hematopoietic Progenitor Cell-Mediated Gene Therapy. Blood. 113: 2172-2180.

    Niiya M, Endo M, Shang D, Zoltick PW, Muvarak NE, Cao WJ, Jin S-Y, Skipwith CG, Motto DG, Flake AW, Zheng XL. Correction of ADAMTS13 Deficiency by In Utero Gene Transfer of Lentiviral Vector encoding ADAMTS13 Genes. Mol Ther. 17(1): 34-41.


    Cuker A, Ptashkin B, Konkle BA, Pipe SW, Whinna HC, Zheng XL, Cines DB, Pollak ES. Interlaboratory agreement in the monitoring of unfractionated heparin using the anti-factor Xa-correlated activated partial thromboplastin time J Thromb Haemost. 10.1111/j.1538-7836.2008.03224.

    Cao WJ, Niiya M, Zheng XW, Shang D, Zheng XL. Inflammatory cytokines inhibit synthesis of ADAMTS13 metalloprotease in hepatic stellate cells and endothelial cells. J Thromb Hemost. 6:1233-1235.

    Zheng XL, Sadler JE. Pathogenesis of Thrombotic Microangiopathies Ann Rev Pathol. 3:249-277.

    Cao WJ, Krishnaswamy S, Camire RM, Lenting PJ, Zheng XL. Factor VIII accelerates proteolytic cleavage of von Willebrand factor by ADAMTS13. Proc Natl Acad Sci USA. 105(21):7416-7421.


    Bennett CL, Kim B, Zakarija A, Bandarenko N, Pandey DK, Buffie CG, McKoy JM, Tevar AD, Cursio JF, Yarnold PR, Kwaan HC, Masi DD, Sarode R, Raife TJ, Kiss JE, Raisch DW, Davidson C, Sadler JE, Ortel TL, Zheng XL, Kato S, Matsumoto M, Uemura M, Fujimura Y. Two Mechanistic Pathways for Thienopyridine-Associated Thrombotic Thrombocytopenic Purpura. J Am Coll Cardiol. 50(12):1138-1143.

    Zhang P, Pan W, Rux AH, Sachais BS, Zheng XL. The cooperative activity between the carboxyl-terminal TSP1 repeats and the CUB domains of ADAMTS13 is crucial for recognition of von Willebrand factor under flow. Blood. 110(6):1887-1894.


    Shelat S, Smith P, Ai J, Zheng XL. Inhibitory autoantibodies against ADAMTS13 in patients with thrombotic thrombocytopenic purpura bind ADAMTS13 protease and may accelerate its clearance in vivo. J Thromb Hemost. 4:1707-1717.

    Shang D, Zheng XW, Niiya M, Zheng XL. Apical sorting of ADAMTS13 in human vascular endothelial cells and Madin-Darby canine kidney cells depend on CUB domains and their association with lipid rafts. Blood. 108(7):2209-2217.

    Cataland SR, Jin M, Zheng XL, George JM, Wu HF. An evaluation of cyclosporine alone for the treatment of early recurrences of thrombotic thrombocytopenic purpura. J Thromb Hemost. 4(5):1162-1164.

    Zheng XL, Sadler JE. Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome Clin. Hematol.

    Niiya M, Uemura M, Zheng XW, Dockal M, Pollak E, Scheiflinger F, Wells R, Zheng XL. Increased ADAMTS13 proteolytic activity in rat hepatic stellate cells upon activation in vitro and in vivo. J Thromb Hemost. 4(5): 1063-1070.



    Shelat S, Ai J, Zheng XL. Molecular Biology of ADAMTS13 and Clinical Utilities of ADAMTS13 Assays. Semin Thromb Hemost. 31 (6): 659-72.


    Ai J, Smith P, Wang S, Zhang P, Zheng XL. The proximal carboxyl terminal domains of ADAMTS13 determine substrate specificity and are all required for cleavage of Von Willebrand factor.  J Biol Chem. 280(33): 29428-34