Doherty D, Lavin M, O’Sullivan JM, et al. Management of elective procedures in low von Willebrand factor patients in the LoVIC study. J Thromb Haemost. December 2020;10.1111

Delaney C, Davizon-Castillo P, Allawzi A, et al. Platelet activation contributes to hypoxia-induced inflammation. Am J Physiol Lung Cell Mol Physiol. ePub December 2020; 10.1152

Porter CC, Di Paola J, Pencheva B. ETV6 Thrombocytopenia and Predisposition to Leukemia. In: Adam MP, Ardinger HH, Pagon RA, et al., eds. GeneReviews®. Seattle (WA): University of Washington, Seattle; November 2020.

Gupta S, Konradt C, Corken A, et al. Hemostasis vs. homeostasis: Platelets are essential for preserving vascular barrier function in the absence of injury or inflammation. Proc Natl Acad Sci U S A. September 2020; 117(39):24316-24325.

Bortot M, Sharifi A, Ashworth K, et al. Pathologic Shear and Elongation Rates Do Not Cause Cleavage of Von Willebrand Factor by ADAMTS13 in a Purified System. Cell Mol Bioeng. July 2020; 13(4):379-390.

Kim DA, Ashworth KJ, Di Paola J, Ku DN. Platelet α-granules are required for occlusive high-shear-rate thrombosis. Blood Adv. July 2020; 4(14):3258-3267

Park I, Johnson LK, Cox A, et al. Hypermethioninemia Leads to Fatal Bleeding and Increased Mortality in a Transgenic I278T Mouse Model of Homocystinuria. Biomedicines. July 2020; 8(8):244.

Di Paola J, Fisher MH. ETV6-related thrombocytopenia and platelet dysfunction. Platelets. ePub May 2020; 32(1):141-143.

Davizon-Castillo P, Allawzi A, Sorrells M, et al. Platelet activation in experimental murine neonatal pulmonary hypertension. Physiol Rep. March 2020; 8(5):e14386.

Botero JP, Lee K, Branchford BR, et al. Glanzmann thrombasthenia: genetic basis and clinical correlates. Haematologica. April 2020; 105(4):888-894.

Link KG, Stobb MT, Sorrells MG, et al. A mathematical model of coagulation under flow identifies factor V as a modifier of thrombin generation in hemophilia A. J Thromb Haemost. February 2020; 18(2):306-317.

Kalot MA, Al-Khatib M, Connell NT, et al. An international survey to inform priorities for new guidelines on von Willebrand disease. Haemophilia. January 2020; 26(1):106-116.


Majtan T, Park I, Cox A, et al. Behavior, body composition, and vascular phenotype of homocystinuric mice on methionine-restricted diet or enzyme replacement therapy. FASEB J. November 2019; 33(11):12477–12486.

Davizon-Castillo P, McMahon B, Aguila S, et al. TNF-α-driven inflammation and mitochondrial dysfunction define the platelet hyperreactivity of aging. Blood. September 2019; 134(9):727–740.

Bortot M, Ashworth K, Sharifi A, et al. Turbulent Flow Promotes Cleavage of VWF (von Willebrand Factor) by ADAMTS13 (A Disintegrin and Metalloproteinase With a Thrombospondin Type-1 Motif, Member 13). Arterioscler Thromb Vasc Biol. September 2019; 39(9):1831–1842.

Di Paola J, Porter CC. ETV6-related thrombocytopenia and leukemia predisposition. Blood. 2019; 134(8):663–667.

Hernandez G, Mills TS, Rabe JL, et al. Pro-inflammatory cytokine blockade attenuates myeloid expansion in a murine model of rheumatoid arthritis. Haematologica. May 2019; haematol.2018.197210

Riemondy KA, Ransom M, Alderman C, et al. Recovery and analysis of transcriptome subsets from pooled single-cell RNA-seq libraries. Nucleic Acids Res. February 2019; 47(4):e20.

Hu Z, Lavik KI, Liu Y, et al. Loss of fibrinogen in zebrafish results in an asymptomatic embryonic hemostatic defect and synthetic lethality with thrombocytopenia. J Thromb Haemost. April 2019; 17(4):607–617.

Aguila S, Lavin M, Dalton N, et al. Increased galactose expression and enhanced clearance in patients with low von Willebrand factor. Blood. April 2019; 133(14):1585–1596.


Di Paola J, Bray PF. One over PAR or one under PAR: vive la différence. Blood. November 2018; 132(19):2007-2008. Kim JS, Kaufman J, Patel SS, Manco-Johnson M, Di Paola J, da Cruz EM. Antiplatelet Effect of Ketorolac in Children After Congenital Cardiac Surgery. World J Pediatr Congenit Heart Surg. November 2018; 9(6):651-658. Link KG, Stobb MT, Di Paola J, Neeves KB, Fogelson AL, Sindi SS, Leiderman K. A local and global sensitivity analysis of a mathematical model of coagulation and platelet deposition under flow. PLoS One. July 2018; 13(7):e0200917. Lavin M, Aguila S, Dalton N, Nolan M, Byrne M, Ryan K, White B, O’Connell NM, O’Sullivan JM, Di Paola J, James PD, O’Donnell JS. Significant gynecological bleeding in women with low von Willebrand factor levels. Blood Adv. July 2018; 2(14):1784-1791. Fisher MH, Di Paola J. Genomics and transcriptomics of megakaryocytes and platelets: Implications for health and disease. Res Pract Thromb Haemost. July 2018; 2(4):630-639. Smith N, Bornikova L, Noetzli L, Guglielmone H, Minoldo S, Backos DS, Jacobson L, Thornburg CD, Escobar M, White-Adams TC, Wolberg AS, Manco-Johnson M, Di Paola J. Identification and characterization of novel mutations implicated in congenital fibrinogen disorders. Res Pract Thromb Haemost. July 2018; 2(4):800-811. Pluthero FG, Di Paola J, Carcao MD, Kahr WHA. NBEAL2 mutations and bleeding in patients with gray platelet syndrome. Platelets. September 2018; 29(6):632-635. Ng CJ, Di Paola J. von Willebrand Disease: Diagnostic Strategies and Treatment Options. Pediatr Clin North Am. June 2018; 65(3):527-541. Shan Z, Liu X, Chen Y, Wang M, Gao YR, Xu L, Dar WA, Lee CG, Elias JA, Castillo PD, Di Paola J, Ju C. Chitinase 3-like-1 promotes intrahepatic activation of coagulation through induction of tissue factor in mice. Hepatology. June 2018; 67(6):2384-2396. Law LA, Graham DK, Di Paola J, Branchford BR. GAS6/TAM Pathway Signaling in Hemostasis and Thrombosis. Front Med (Lausanne). May 2018; 5:137. Ng CJ, McCrae KR, Ashworth K, Sosa LJ, Betapudi V, Manco-Johnson MJ, Liu A, Dong JF, Chung D, White-Adams TC, López JA, Di Paola J. Effects of anti-β2GPI antibodies on VWF release from human umbilical vein endothelial cells and ADAMTS13 activity. Res Pract Thromb Haemost. March 2018; 2(2):380–389. Branchford BR, Stalker TJ, Law L, Acevedo G, Sather S, Brzezinski C, Wilson KM, Minson K, Lee-Sherick AB, Davizon-Castillo P, Ng C, Zhang W, Neeves KB, Lentz SR, Wang X, Frye SV, Shelton Earp H 3rd, DeRyckere D, Brass LF, Graham DK, Di Paola JA. The small-molecule MERTK inhibitor UNC2025 decreases platelet activation and prevents thrombosis. Journal of Thrombosis and Haemostasis. February 2018; 16(2):352-363. Lehmann M, Ashworth K, Manco-Johnson M, Di Paola J, Neeves KB, Ng CJ. Evaluation of a microfluidic flow assay to screen for von Willebrand disease and low von Willebrand factor levels. Journal of Thrombosis and Haemostasis. January 2018; 16(1):104-115.


McGraw MD, Osborne CM, Mastej EJ, Di Paola JA, Anderson DR, Holmes WW, Paradiso DC, Garlick RB, Hendry-Hofer TB, Rancourt RC, Smith RW, Burns C, Roe GB, Rioux JS, White CW, Veress LA. Editor’s Highlight: Pulmonary Vascular Thrombosis in Rats Exposed to Inhaled Sulfur Mustard. Toxicol Sci. October 2017; 159(2):461-469. Lavin M, Aguila S, Schneppenheim S, Dalton N, Jones KL, O’Sullivan JM, O’Connell NM, Ryan K, White B, Byrne M, Rafferty M, Doyle MM, Nolan M, Preston RJS, Budde U, James P, Di Paola J, O’Donnell JS. Novel insights into the clinical phenotype and pathophysiology underlying low VWF levels. Blood. November 2017; 130(21):2344-2353. Miller CH, Boylan B, Shapiro AD, Lentz SR, Wicklund BM; Hemophilia Inhibitor Research Study Investigators. Limit of detection and threshold for positivity of the Centers for Disease Control and Prevention assay for factor VIII inhibitors. J Thromb Haemost. October 2017; 15(10):1971-1976. Kastelowitz N, Tamura R, Onasoga A, Stalker TJ, White OR, Brown PN, Brodsky GL, Brass LF, Branchford BR, Di Paola J, Yin H. Peptides derived from MARCKS block coagulation complex assembly on phosphatidylserine. Sci Rep. June 2017; 7(1):4275. Davizon-Castillo P, Di Paola J. Tamoxifen Suppresses Platelet Activation- Supported Angiogenesis and Metastasis. Arterioscler Thromb Vasc Biol. April 2017; 37(4):611-612. Maloney JP, Branchford BR, Brodsky GL, Cosmic MS, Calabrese DW, Aquilante CL, Maloney KW, Gonzalez JR, Zhang W, Moreau KL, Wiggins KL, Smith NL, Broeckel U, Di Paola J. The ENTPD1 promoter polymorphism -860 A > G (rs3814159) is associated with increased gene transcription, protein expression, CD39/NTPDase1 enzymatic activity, and thromboembolism risk. FASEB J. July 2017; 31(7):2771-2784. Moreira DC, Di Paola J. Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome: reply. J Thromb Haemost. April 2017; 15(4):827-828. Schoeman RM, Rana K, Danes N, Lehmann M, Di Paola JA, Fogelson AL, Leiderman K, Neeves KB. A microfluidic model of hemostasis sensitive to platelet function and coagulation. Cell Mol Bioeng. February 2017; 10(1):3-15.


Moreira DC, Ng CJ, Quinones R, Liang X, Chung DW, Di Paola J. Microangiopathic hemolytic anemia due to ADAMTS-13 loss in idiopathic systemic capillary leak syndrome. J Thromb Haemost. December 2016; 14(12):2353-2355. Deng W, Xu Y, Chen W, Paul DS, Syed AK, Dragovich MA, Liang X, Zakas P, Berndt MC, Di Paola J, Ware J, Lanza F, Doering CB, Bergmeier W, Zhang XF, Li R. Platelet clearance via shear-induced unfolding of a membrane mechanoreceptor. Nat Commun. September 2016; 7:12863. Boylan B, Rice AS, Neff AT, Manco-Johnson MJ, Kempton CL, Miller CH; Hemophilia Inhibitor Research Study Investigators. Survey of the anti-factor IX immunoglobulin profiles in patients with hemophilia B using a fluorescence-based immunoassay. J Thromb Haemost. October 2016; 14(10):1931-1940. Gupta S, Heiman M, Duncan N, Hinckley J, Di Paola J, Shapiro AD. Variable bleeding phenotype in an Amish pedigree with von Willebrand disease. Am J Hematol. October 2016; 91(10):E431-5. White-Adams TC, Ng CJ, Jacobi PM, Haberichter SL, Di Paola JA. Mutations in the D’D3 region of VWF traditionally associated with type 1 VWD lead to quantitative and qualitative deficiencies of VWF. Thromb Res. September 2016; 145:112-8. Guglielmone HA, Smith N, Minoldo S, Jarchum GD, White-Adams T, Di Paola J. Platelet abnormalities in a family with von Willebrand disease type 2B (V1316M) and association with bleeding score. Haemophilia. September 2016; 22(5):e455-8. Tomberg K, Khoriaty R, Westrick RJ, Fairfield HE, Reinholdt LG, Brodsky GL, Davizon-Castillo P, Ginsburg D, Di Paola J. Spontaneous 8bp Deletion in Nbeal2 Recapitulates the Gray Platelet Syndrome in Mice. PLoS One. March 2016; 11(3):e0150852. Othman M, Kaur H, Favaloro EJ, Lillicrap D, Di Paola J, Harrison P, Gresele P; Subcommittees on von Willebrand Disease and Platelet Physiology. Platelet type von Willebrand disease and registry report: communication from the SSC of the ISTH. J Thromb Haemost. February 2016; 14(2):411-4.


Di Paola J. Paris-Trousseau: evidence keeps pointing to FLI1. Blood. October 2015; 126(17):1973-4. Kirkpatrick G, Noetzli L, Di Paola J, Porter CC. ETV6 mutations define a new cancer predisposition syndrome. Oncotarget. July 2015; 6(19):16830-1. Chen J, Hinckley JD, Haberichter S, Jacobi P, Montgomery R, Flood VH, Wong R, Interlandi G, Chung DW, López JA, Di Paola J. Variable content of von Willebrand factor mutant monomer drives the phenotypic variability in a family with von Willebrand disease. Blood. July 2015; 126(2):262-9. Branchford BR, Ng CJ, Neeves KB, Di Paola J. Microfluidic technology as an emerging clinical tool to evaluate thrombosis and hemostasis. Thromb Res. July 2015; 136(1):13-9. Bodó I, Eikenboom J, Montgomery R, Patzke J, Schneppenheim R, Di Paola J; von Willebrand factor Subcommittee of the Standardization and Scientific Committee of the International Society for Thrombosis and Haemostasis. Platelet- dependent von Willebrand factor activity.Nomenclature and methodology: communication from the SSC of the ISTH. J Thromb Haemost. July 2015; 13(7):1345-50. Boylan B, Rice AS, De Staercke C, Eyster ME, Yaish HM, Knoll CM, Bean CJ, Miller CH; Hemophilia Inhibitor Research Study Investigators. Evaluation of von Willebrand factor phenotypes and genotypes in Hemophilia A patients with and without identified F8 mutations. J Thromb Haemost. June 2015; 13(6):1036-42. Noetzli L, Lo RW, Lee-Sherick AB, Callaghan M, Noris P, Savoia A, Rajpurkar M, Jones K, Gowan K, Balduini C, Pecci A, Gnan C, De Rocco D, Doubek M, Li L, Lu L, Leung R, Landolt-Marticorena C, Hunger S, Heller P, Gutierrez-Hartmann A, Xiayuan L, Pluthero FG, Rowley JW, Weyrich AS, Kahr WHA, Porter CC, Di Paola J. Germline mutations in ETV6 are associated with thrombocytopenia, red cell macrocytosis and predisposition to lymphoblastic leukemia. Nat Genet. May 2015; 47(5):535-538. Ng C, Motto DG, Di Paola J. Diagnostic approach to von Willebrand disease. Blood. March 2015; 125(13):2029-37. Boylan B, Rice AS, Dunn AL, Tarantino MD, Brettler DB, Barrett JC, Miller CH; Hemophilia Inhibitor Research Study Investigators; Hemophilia Inhibitor Research Study Investigators. Characterization of the anti-factor VIII immunoglobulin profile in patients with hemophilia A by use of a fluorescence- based immunoassay. J Thromb Haemost. January 2015; 13(1):47-53.


Hinckley J, Di Paola J. Genetic basis of congenital platelet disorders. Hematology Am Soc Hematol Educ Program. December 2014; 2014(1):337-42. Gonzalez-Alegre P, Di Paola J, Wang K, Fabbro S, Yu HC, Shaikh TH, Darbro BW, Bassuk AG. Evaluating Familial Essential Tremor with Novel Genetic Approaches: Is it a Genotyping or Phenotyping Issue?. Tremor Other Hyperkinet Mov (N Y). October 2014; 4:258. Di Paola J. Understanding congenital platelet disorders. Clin Adv Hematol Oncol. July 2014; 12(7):461-3. Aleman MM, Byrnes JR, Wang JG, Tran R, Lam WA, Di Paola J, Mackman N, Degen JL, Flick MJ, Wolberg AS. Factor XIII activity mediates red blood cell retention in venous thrombi. J Clin Invest. August 2014; 124(8):3590-600. Boulden Warren B, Di Paola JA. Platelets join the world of “Omics”. Blood. April 2014; 123(16):2446-7. Branchford BR, Ruegg K, Villalobos-Menuey E, Jacobson LJ, Di Paola JA, Manco-Johnson M. FVIII/VWF ratio is not a reliable predictor of VWD in children. Pediatr Blood Cancer. May 2014; 61(5):936-9. Soucie JM, Miller CH, Kelly FM, Payne AB, Creary M, Bockenstedt PL, Kempton CL, Manco-Johnson MJ, Neff AT; Haemophilia Inhibitor Research Study Investigators. A study of prospective surveillance for inhibitors among persons with haemophilia in the United States. Haemophilia. March 2014; 20(2):230-7.


Hinckley JD, Abbott D, Burns TL, Heiman M, Shapiro AD, Wang K, Di Paola J. Quantitative trait locus linkage analysis in a large Amish pedigree identifies novel candidate loci for erythrocyte traits. Mol Genet Genomic Med. September 2013; 1(3):131-141.

Kahr WH, Lo RW, Li L, Pluthero FG, Christensen H, Ni R, Vaezzadeh N, Hawkins CE, Weyrich AS, Di Paola J, Landolt-Marticorena C, Gross PL. Abnormal megakaryocyte development and platelet function in Nbeal2(-/-) mice. Blood. November 2013; 122(19):3349-58.

Miller CH, Rice AS, Boylan B, Shapiro AD, Lentz SR, Wicklund BM, Kelly FM, Soucie JM; Hemophilia Inhibitor Research Study Investigators. Comparison of clot-based, chromogenic and fluorescence assays for measurement of factor VIII inhibitors in the US Hemophilia Inhibitor Research Study. J Thromb Haemost. July 2013; 11(7):1300-9.

Di Paola J. SHPing in different directions in platelet production. Blood. May 2013; 121(20):4018-9.

Mannuccio Mannucci P, Kyrle PA, Schulman S, Di Paola J, Schneppenheim R, Cox Gill J. Prophylactic efficacy and pharmacokinetically guided dosing of a von Willebrand factor/factor VIII concentrate in adults and children with von Willebrand’s disease undergoing elective surgery: a pooled and comparative analysis of data from USA and European Union clinical trials. Blood Transfus. October 2013; 11(4):533-40.

Flood VH, Friedman KD, Gill JC, Haberichter SL, Christopherson PA, Branchford BR, Hoffmann RG, Abshire TC, Dunn AL, Di Paola JA, Hoots WK, Brown DL, Leissinger C, Lusher JM, Ragni MV, Shapiro AD, Montgomery RR. No increase in bleeding identified in type 1 VWD subjects with D1472H sequence variation. Blood. May 2013; 121(18):3742-4.

Branchford BR, Monahan PE, Di Paola J. New developments in the treatment of pediatric hemophilia and bleeding disorders. Curr Opin Pediatr. February 2013; 25(1):23-30.

Neeves KB, Onasoga AA, Hansen RR, Lilly JJ, Venckunaite D, Sumner MB, Irish AT, Brodsky G, Manco-Johnson MJ, Di Paola JA. Sources of variability in platelet accumulation on type 1 fibrillar collagen in microfluidic flow assays. PLoS One. 2013; 8(1):e54680.

Ahmad S, Ahmad A, Rancourt RC, Neeves KB, Loader JE, Hendry-Hofer T, Di Paola J, Reynolds SD, White CW. Tissue factor signals airway epithelial basal cell survival via coagulation and protease-activated receptor isoforms 1 and 2. Am J Respir Cell Mol Biol. January 2013; 48(1):94-104.


Marchi R, Walton BL, McGary CS, Lin FC, Ma AD, Pawlinski R, Mackman N, Campbell RA, Di Paola J, Wolberg AS. Dysregulated coagulation associated with hypofibrinogenaemia and plasma hypercoagulability: implications for identifying coagulopathic mechanisms in humans. Thromb Haemost. September 2012; 108(3):516-26.

Miller CH, Platt SJ, Rice AS, Kelly F, Soucie JM; Hemophilia Inhibitor Research Study Investigators. Validation of Nijmegen-Bethesda assay modifications to allow inhibitor measurement during replacement therapy and facilitate inhibitor surveillance. J Thromb Haemost. June 2012; 10(6):1055-61.

Branchford BR, Di Paola J. Making a diagnosis of VWD. Hematology Am Soc Hematol Educ Program. 2012; 2012:161-7.

Miller CH, Benson J, Ellingsen D, Driggers J, Payne A, Kelly FM, Soucie JM, Craig Hooper W; Hemophilia Inhibitor Research Study Investigators. F8 and F9 mutations in US haemophilia patients: correlation with history of inhibitor and race/ethnicity. Haemophilia. May 2012; 18(3):375-82.


Hansen RR, Tipnis AA, White-Adams TC, Di Paola JA, Neeves KB. Characterization of collagen thin films for von Willebrand factor binding and platelet adhesion. Langmuir. November 2011; 27(22):13648-58.

Di Paola J, Johnson J. Thrombocytopenias due to gray platelet syndrome or THC2 mutations. Semin Thromb Hemost. September 2011; 37(6):690-7.

Kahr WH, Hinckley J, Li L, Schwertz H, Christensen H, Rowley JW, Pluthero FG, Urban D, Fabbro S, Nixon B, Gadzinski R, Storck M, Wang K, Ryu GY, Jobe SM, Schutte BC, Moseley J, Loughran NB, Parkinson J, Weyrich AS, Di Paola J. Mutations in NBEAL2, encoding a BEACH protein, cause gray platelet syndrome. Nat Genet. July 2011; 43(8):738-40.

Kessler CM, Friedman K, Schwartz BA, Gill JC, Powell JS; Wilate PK Study Investigators. The pharmacokinetic diversity of two von Willebrand factor (VWF)/ factor VIII (FVIII) concentrates in subjects with congenital von Willebrand disease.Results from a prospective, randomised crossover study. Thromb Haemost. August 2011; 106(2):279-88.

Di Paola J, Lethagen S, Gill J, Mannucci P, Manco-Johnson M, Bernstein J, Nichols WL, Bergman GE. Presurgical pharmacokinetic analysis of a von Willebrand factor/factor VIII (VWF/FVIII) concentrate in patients with von Willebrand’s disease (VWD) has limited value in dosing for surgery. Haemophilia. September 2011; 17(5):752-8.

Fabbro S, Kahr WH, Hinckley J, Wang K, Moseley J, Ryu GY, Nixon B, White JG, Bair T, Schutte B, Di Paola J. Homozygosity mapping with SNP arrays confirms 3p21 as a recessive locus for gray platelet syndrome and narrows the interval significantly. Blood. March 2011; 117(12):3430-4.

DiPaola J, Lillicrap D. Challenges and innovations in the treatment of bleeding disorders. Thromb Res. January 2011; 127 Suppl 1:S1-2.


Monahan PE, Di Paola J. Recombinant factor IX for clinical and research use. Semin Thromb Hemost. July 2010; 36(5):498-509.

James P, Di Paola J. The application of genetics to inherited bleeding disorders. Haemophilia. July 2010; 16 Suppl 5:35-9.

Björkman S, Blanchette VS, Fischer K, Oh M, Spotts G, Schroth P, Fritsch S, Patrone L, Ewenstein BM; Advate Clinical Program Group, Collins PW. Comparative pharmacokinetics of plasma- and albumin-free recombinant factor VIII in children and adults: the influence of blood sampling schedule on observed age-related differences and implications for dose tailoring. J Thromb Haemost. April 2010; 8(4):730-6.

Flood VH, Gill JC, Morateck PA, Christopherson PA, Friedman KD, Haberichter SL, Branchford BR, Hoffmann RG, Abshire TC, Di Paola JA, Hoots WK, Leissinger C, Lusher JM, Ragni MV, Shapiro AD, Montgomery RR. Common VWF exon 28 polymorphisms in African Americans affecting the VWF activity assay by ristocetin cofactor. Blood. July 2010; 116(2):280-6.


Metjian AD, Wang C, Sood SL, Cuker A, Peterson SM, Soucie JM, Konkle BA; HTCN Study Investigators. Bleeding symptoms and laboratory correlation in patients with severe von Willebrand disease. Haemophilia. July 2009; 15(4):918-25.

Collins PW, Blanchette VS, Fischer K, Björkman S, Oh M, Fritsch S, Schroth P, Spotts G, Astermark J, Ewenstein B; rAHF-PFM Study Group. Break-through bleeding in relation to predicted factor VIII levels in patients receiving prophylactic treatment for severe hemophilia A. J Thromb Haemost. March 2009; 7(3):413-20.


Young G, Ebbesen LS, Viuff D, Di Paola J, Konkle BA, Negrier C, Pasi J, Ingerslev J. Evaluation of thromboelastography for monitoring recombinant activated factor VII ex vivo in haemophilia A and B patients with inhibitors: a multicentre trial. Blood Coagul Fibrinolysis. June 2008; 19(4):276-82.

Blanchette VS, Shapiro AD, Liesner RJ, Hernández Navarro F, Warrier I, Schroth PC, Spotts G, Ewenstein BM; rAHF-PFM Clinical Study Group. Plasma and albumin-free recombinant factor VIII: pharmacokinetics, efficacy and safety in previously treated pediatric patients. J Thromb Haemost. August 2008; 6(8):1319-26.

Abbott D, Di Paola J. VWD type 1: a calculated diagnosis. Blood. April 2008; 111(8):3919-20.

Sharthkumar A, Greist A, Di Paola J, Winay J, Roberson C, Heiman M, Herbert S, Parameswaran R, Shapiro A. Biologic response to subcutaneous and intranasal therapy with desmopressin in a large Amish kindred with Type 2M von Willebrand disease. Haemophilia. May 2008; 14(3):539-48.

Hoots WK, Ebbesen LS, Konkle BA, Auerswald GK, Roberts HR, Weatherall J, Ferran JM, Ljung RC; Novoseven (F7HAEM-1505) Investigators. Secondary prophylaxis with recombinant activated factor VII improves health-related quality of life of haemophilia patients with inhibitors. Haemophilia. May 2008; 14(3):466-75.

Jobe SM, Wilson KM, Leo L, Raimondi A, Molkentin JD, Lentz SR, Di Paola J. Critical role for the mitochondrial permeability transition pore and cyclophilin D in platelet activation and thrombosis. Blood. February 2008; 111(3):1257-65.


Di Paola J, Jugessur A, Goldman T, Reiland J, Tallman D, Sayago C, Murray JC. Platelet glycoprotein I(b)alpha and integrin alpha2 beta1 polymorphisms: gene frequencies and linkage disequilibrium in a population diversity panel. J Thromb Haemost. July 2005; 3(7):1511-21.

Shapiro AD, Di Paola J, Cohen A, Pasi KJ, Heisel MA, Blanchette VS, Abshire TC, Hoots WK, Lusher JM, Negrier C, Rothschild C, Roth DA. The safety and efficacy of recombinant human blood coagulation factor IX in previously untreated patients with severe or moderately severe hemophilia B. Blood. January 2005; 105(2):518-25.


Di Paola J. Product selection issues in the management of hemophilia B. Blood Coagul Fibrinolysis. June 2004; 15 Suppl 2:S17-8.

Jobe S, Donohoue P, Di Paola J. Deep venous thrombosis and Turner syndrome. J Pediatr Hematol Oncol. April 2004; 26(4):272.

Di Paola J, Goldman T, Qian Q, Patil SR, Schutte BC. Breakpoint of a
balanced translocation (X:14) (q27.1;q32.3) in a girl with severe hemophilia B
maps proximal to the factor IX gene
. J Thromb Haemost. April 2004; 2(4):682.


Leo L, Di Paola J, Judd BA, Koretzky GA, Lentz SR. Role of the adapter protein SLP-76 in GPVI-dependent platelet procoagulant responses to collagen. Blood. October 2002; 100(8):2839-44.

Di Paola JA, Buchanan GR. Immune thrombocytopenic purpura. Pediatr Clin North Am. October 2002; 49(5):911-28.

Prior to 2002

Di Paola J, Nugent D, Young G. Current therapy for rare factor deficiencies. Haemophilia. January 2001; 7 Suppl 1:16-22.

Di Paola J, Federici AB, Mannucci PM, Canciani MT, Kritzik M, Kunicki TJ,
Nugent D. Low platelet alpha2beta1 levels in type I von Willebrand disease
correlate with impaired platelet function in a high shear stress system
. Blood. June 1999; 93(11):3578-82.

Doncel GF, Di Paola JA, Lustig L. Sequential study of the histopathology and
cellular and humoral immune response during the development of an autoimmune
orchitis in Wistar rats
. Am J Reprod Immunol. June 1989; 20(2):44-51.