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Classification of von willebrand disease

1987; Wiley; Volume: 1; Issue: 4 Linguagem: Inglês

10.1002/jcla.1860010408

1098-2825

Zaverio M. Ruggeri, T S Zimmerman,

Coagulation, Bradykinin, Polyphosphates, and Angioedema

Journal of Clinical Laboratory AnalysisVolume 1, Issue 4 p. 353-362 Review Article Classification of von willebrand disease Zaverio M. Ruggeri, Corresponding Author Zaverio M. Ruggeri Division of Experimental Hemostasis, Department of Basic and Clinical Research, Scripps Clinic and Research Foundation, La JoHa, CaliforniaZayerio M. Ruggeri, MD (BCR8), Scripps Clinic and Research Foundation, 10666 N. Torrey Pines Road, La Jolla. CA92037Search for more papers by this authorTheodore S. Zimmerman, Theodore S. Zimmerman Division of Experimental Hemostasis, Department of Basic and Clinical Research, Scripps Clinic and Research Foundation, La JoHa, CaliforniaSearch for more papers by this author Zaverio M. Ruggeri, Corresponding Author Zaverio M. Ruggeri Division of Experimental Hemostasis, Department of Basic and Clinical Research, Scripps Clinic and Research Foundation, La JoHa, CaliforniaZayerio M. Ruggeri, MD (BCR8), Scripps Clinic and Research Foundation, 10666 N. Torrey Pines Road, La Jolla. CA92037Search for more papers by this authorTheodore S. Zimmerman, Theodore S. Zimmerman Division of Experimental Hemostasis, Department of Basic and Clinical Research, Scripps Clinic and Research Foundation, La JoHa, CaliforniaSearch for more papers by this author First published: 1987 https://doi.org/10.1002/jcla.1860010408Citations: 7AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat References 1 von Willebrand EA: Hereditär pseudohemofili. Finska Lakaresallskapets Handlingar, 67: 7–112, 1926. 2 Zimmerman TS, Ratnoff, OD, Powell AE: Immunologie differentiation of classic hemophilia (Factor VIII deficiency) and von Wille-brand's disease. With observations on combined deficiencies of antihemophilic factor and proaccelerin (Factor V) and on an acquired circulating anticoagulant against antihemophilic factor. J Clin Invest 40: 244–254, 1971. 3 Jaffe EA, Hoyer LW, Nachman RL: Synthesis of von Willebrand factor by cultured human endothelial cells. Proc Natl Acad Sci USA 71: 1906 1909, 1974. 4 Sporn LA, Chavin SI, Marder VJ, Wagner DD: Biosynthesis of von Willebrand protein by human megakaryocytes. J Clin Invest 76: 1102–1106, 1985. 5 Ginsburg D, Handin RI, Bonthron DT, Donion TA, Bruns GA, Latt SA, Orkin SH: Human von Willebrand factor (vWF): Isolation of complementary DNA (cDNA) clones and chromosomal localization. Science 228: 1401–1406, 1985. 6 Verweij CL, de Vries CJM, Distel B, van Zonneveld A-J, von Kessel AG, Van Mourik JA, Pannekoek H: Construction of cDNA coding for human von Willebrand factor using antibody probes for colony-screening and mapping of the chromosomal gene. Nucleic Acids Res 13: 4699–4717, 1985. 7 Bonthron D, Orr EC, Mitsock LM, Ginsburg D, Handin RI, Orkin SH: Nucleotide sequence of pre-pro-von Willebrand factor cDNA. Nucleic Acids Res 14: 7125–7127, 1986. 8 Fowler WE, Fretto LJ, Hamilton KK, Erickson HP, McKee PA: Substructure of human von Willebrand factor. J Clin Invest 76: 1491–1500, 1985. 9 Wagner DD, Marder VJ: Biosynthesis of von Willebrand protein by human endothelial cells: Processing steps and their intracellular localization. J Cell Biol 99: 2123–2130, 1984. 10 Fay PJ, Kawai Y, Wagner DD, Ginsburg D, Bonthron D, Ohlsson- Wilhelm BM, Chavin SI, Abraham GN, Handin RI, Orkin SH, Montgomery RR, Marder VJ: Propolypeptide of von Willebrand factor circulates in blood and is identical to von Willebrand antigen II. Science 232: 995–998, 1986. 11 McCarroll DR, Levin EG, Montgomery RR: Endothelial cell synthesis of von Willebrand antigen II, von Willebrand factor, and von Willebrand factor/von Willebrand antigen II complex. J Clin Invest 75: 1089–1095, 1985. 12 Scott JP, Montgomery RR: Platelet von Willebrand's antigen II: active release by aggregating agents and a marker of platelet release reaction in vivo. Blood 58: 1075–1080, 1981. 13 Montgomery RR, Zimmerman TS: von Willebrand's disease antigen II. A new plasma and platelet antigen deficient in severe von Willebrand's disease. J Clin Invest 61: 1498–1507, 1978. 14 Titani K, Kumar S, Takio K, Ericsson LH, Wade RD, Ashida K, Walsh KA, Chopek MW, Sadler JE, Fujikawa K: Amino acid sequence of human von Willebrand factor. Biochemistry 25: 3171–3184, 1986. 15 Lynch DC, Zimmerman TS, Collins CJ, Brown M, Morin MJ, Ling EH, Livingston DM: Molecular cloning of cDNA for human von Willebrand factor: Authentication by a new method. Cell 41: 49–56, 1985. 16 Sadler JE, Shelton-Inloes BB, Sorace JM, Harlan JM, Titani K, Davie EW: Cloning and characterization of two cDNAs coding for human von Willebrand factor. Proc Natl Acad Sci USA 82: 6394–6398, 1985. 17 Wagner DD, Mayadas T, Marder VJ: Initial glycosylation and acidic pH in the Golgi apparatus are required for multimerization of von Willebrand factor. J Cell Biol 102: 1320–1324, 1986. 18 Lynch DC, Zimmerman TS, Kirby EP, Livingston DM: Subunit composition of oligomeric human von Willebrand factor. J Biol Chem 258: 12757–12760, 1983. 19 Wagner DD, Marder VJ: Biosynthesis of von Willebrand protein by human endothelial cells: Identification of a large precursor polypeptide chain. J Biol Chem 258: 2065–2067, 1983. 20 Montgomery RR, Dent J, Schmidt W, Kyrle P, Niessner H, Ruggeri ZM, Zimmerman TS: Hereditary persistence of circulating pro von Willebrand factor (pro-vWF). Circulation 74: II-406a, 1986. 21 Loesberg C, Gonsalves MD, Zandbergen J, Willems C, Van Aken WG, Stel HV, Van Mourik JA, DeGroot PG: The effect of calcium on the secretion of factor VIII-related antigen by cultured human endothelial cells. Biochim Biophys Acta 763: 160–168, 1983. 22 Sporn LA, Marder VJ, Wagner DD: Inducible secretion of large, biologically potent von Willebrand factor multimers. Cell 46: 185–190, 1986. 23 Sussman II, Rand JH: Subendothelial deposition of von Willebrand's factor requires the presence of endothelial cells. J Lab Clin Med 100: 526–532, 1982. 24 Rand JH, Sussman II, Gordon RE, Chu SV, Solomon V: Localization of factor-VIII-related antigen in human vascular subendothelium. Blood 55: 752–756, 1980. 25 Zucker MB, Broekman MJ, Kaplan KL: Factor VIII-related antigen in human blood platelets. Localization and release by thrombin and collagen. J Lab Clin Med 94: 675–682, 1979. 26 Koutts J, Walsh PN, Plow EF, Fenton JW, Bouma BN, Zimmerman TS: Active release of human platelet Factor VIII-related antigen by adenosine diphosphate, collagen and thrombin. J Clin Invest 62: 1255–1263, 1978. 27 Tschopp TB, Weiss HJ, Baumgartner HR: Decreased adhesion of platelets to subendothelium in von Willebrand's disease. J Lab Clin Med 83: 296–300, 1974. 28 Turitto VT, Weiss HJ, Baumgartner HR: Platelet interaction with rabbit subendothelium in von Willebrand's disease: Altered thrombus formation distinct from defective platelet adhesion. J Clin Invest 74: 1730–1741, 1984. 29 Vehar GA, Davie EW: Preparation and properties of bovine factor VIII (antihemophilic factor). Biochemistry 19: 401–410, 1980. 30 Fulcher CA, Zimmerman TS: Characterization of the human factor VIII procoagulant protein with a heterologous precipitating antibody. Proc Natl Acad Sci USA 79: 1648–1652, 1982. 31 Weiss HJ, Sussman II, Hoyer LW: Stabilization of factor VIII in plasma by the von Willebrand factor. Studies on posttransfusion and dissociated factor VIII and in patients with von Willebrand's disease. J Clin Invest 60: 390–404, 1977. 32 Ruggeri ZM, De Marco L, Gatti L. Bader R, Montgomery RR: Platelets have more than one binding site for von Willebrand factor. J Clin Invest 72: 1–12, 1983. 33 Fujimura Y, Titani K, Holland LZ, Russell SR, Roberts JR, Elder JH, Ruggeri ZM, Zimmerman TS: von Willebrand factor. A reduced and alkylated 52/48-kDa fragment beginning at amino acid residue 449 contains the domain interacting with platelet glycoprotein Ib. J Biol Chem 261: 381–385, 1986. 34 Fujimura Y, Titani K, Holland LZ, Roberts JR, Kostel P, Ruggeri ZM, Zimmerman TS: A heparin-binding domain of human von Willebrand factor. Characterization and localization to a tryptic fragment extending from amino acid residue Val-449 to Lys-728. J Biol Chem 262: 1734–1739, 1987. 35 Pareti FI, Fujimura Y, Dent JA, Holland LZ, Zimmerman TS, Ruggeri ZM: Isolation and characterization of a collagen binding domain in human von Willebrand factor. J Biol Chem 261: 15310–15315, 1986. 36 Plow EF, Pierschbacher MD, Ruoslahti E, Marguerie GA, Ginsberg MH: The effect of Arg-Gly-Asp-containing peptides on fibrinogen and von Willebrand factor binding to platelets. Proc Natl Acad Sci USA 82: 8057–8061, 1985. 37 Doolittle RF, Watt KWK, Colltrell BA, Strong DD, Riley M: The amino acid sequence of the α-chain of human fibrinogen. Nature 280: 464–468, 1979. 38 Pierschbacher MD, Ruoslahti E: Variants of the cell recognition site of fibronectin that retain attachment-promoting activity. Proc Natl Acad Sci USA 81: 5985–5988, 1984. 39 Fretto LJ, Fowler WE, McCaslin DR, Erickson HP, McKee PA: Substructure of human von Willebrand factor. Proteolysis by V8 and characterization of two functional domains. J Biol Chem 261: 15679–15689, 1986. 40 Kalafatis M, Takahashi Y, Girma JP, Meyer D: A monomeric von Willebrand factor fragment (Spl) located between amino acid residues 911 and 1365 contains a domain interacting with collagen. Blood 68: 348a, 1986. 41 Roth GJ, Titani K, Hoyer LW, Hickey MJ: Localization of binding sites within human von Willebrand factor for monomeric Type III collagen. Biochemistry 25: 8357–8361, 1986. 42 Foster PA, Fulcher CA, Marti T, Titani K, Zimmerman TS: A major factor VIII binding domain resides within the amino-terminal 272 amino acid residues of von Willebrand factor. J Biol Chem 262: 8443, 1987. 43 De Marco L, Girolami A, Russell S, Ruggeri ZM: Interaction of asialo von Willebrand factor with glycoprotein Ib induces fibrinogen binding to the glycoprotein IIb/IIIa complex and mediates platelet aggregation. J Clin Invest 75: 1198–1203, 1985. 44 De Marco L, Girolami A, Zimmerman TS, Ruggeri ZM: Interaction of purified IIB von Willebrand factor with the platelet membrane glycoprotein Ib induces fibrinogen binding to the glycoprotein IIb/IIIa complex and initiates aggregation. Proc Natl Acad Sci USA 82: 7424–7428, 1985. 45 Gralnick HR, Williams SB, Coller BS: Asialo von Willebrand factor interactions with platelets. Interdependence of glycoproteins Ib and IIb/IIIa for binding and aggregation. J Clin Invest 75: 19–25, 1985. 46 Marguerie GA, Plow EF, Edgington TS: Human platelets possess an inducible and saturable receptor specific for fibrinogen. J Biol Chem 254: 5357–5363, 1979. 47 De Marco L, Girolami A, Zimmerman TS, Ruggeri ZM: von Willebrand factor interaction with the glycoprotein IIb/IIIa complex. Its role in platelet function as demonstrated in patients with congenital afibrinogenemia. J Clin Invest 77: 1272–1277, 1986. 48 Dixit VM, Haverstick DM, O'Rourke K, Hennessy SW, Broekelmann TJ, McDonald JA, Grant GA, Santoro SA, Frazier WA: Inhibition of platelet aggregation by a monoclonal antibody against human fibronectin. Proc Natl Acad Sci USA 82: 3844–3848, 1985. 49 Sakariassen KS, Nievelstein PF, Coller BS, Sixma JJ: The role of platelet membrane glycoproteins Ib and IIb-IIIa in platelet adherence to human artery subendothelium. Br J Haematol 63: 681–691, 1986. 50 Hoyer LW, Rizza CR, Tuddenham EG, Carta CA, Armitage H, Rotblat F: Von Willebrand factor multimer patterns in von Willebrand's disease. Br J Haematol 55: 493–507, 1983. 51 Ciavarella G, Ciavarella N, Antoncecchi S, De Mattia D, Ranieri P, Dent J, Zimmerman TS, Ruggeri ZM: High-resolution analysis of von Willebrand factor multimeric compositin defines a new variant of type I von Willebrand disease with aberrant structure but presence of all size multimers (type IC). Blood 66: 1423–1429, 1985. 52 Mannucci PM, Lombardi R, Bader R, Vianello L, Federici AB, Solinas S, Mazzucconi MG, Mariani G: Heterogeneity of type I von Willebrand disease: Evidence for a subgroup with an abnormal von Willebrand factor. Blood 66: 796–802, 1985. 53 Weiss HJ, Pietu G, Rabinowitz R, Girma JP, Rogers J, Meyer D: Heterogeneous abnormalities in the multimeric structure, antigenic properties, and plasma-platelet content of factor VIII/von Willebrand factor in subtypes of classic (type I) and variant (type IIA) von Willebrand's disease. J Lab Clin Med 101: 411–425, 1983. 54 Weiss HJ, Sussman II: A new von Willebrand variant ( type I, New York): increased ristocetin-induced platelet aggregation and plasma von Willebrand factor containing the full range of multimers. Blood 68: 149–156, 1986. 55 Holmberg L, Berntorp E, Donner M, Nilsson IM: von Willebrand's disease characterized by increased ristocetin sensitivity and the presence of all von Willebrand factor multimers in plasma. Blood 68: 668–672, 1986. 56 Mannucci PM, Lombardi R, Rodeghiero F, Castman G, Federici AB: A new variant of von Willebrand disease (vWD) with larger than normal high-molecular-weight (HMW) von Willebrand factor (vWF) multimers. Blood 68: 338a, 1986. 57 Ruggeri ZM, Zimmerman TS: Variant von Willebrand's disease: Characterization of two subtypes by analysis of multimeric composition of factor VIII/von Willebrand factor in plasma and platelets. J Clin Invest 65: 1318–1325, 1980. 58 Gralnick HR, Williams SB, McKeown LP, Maisonneuve P, Jenneau C, Sultan Y, Rick ME: In vitro correction of the abnormal multimeric structure of von Willebrand factor in type IIA von Willebrand disease. Proc Natl Acad Sci USA 82: 5968–5972, 1985. 59 Batlle J, Lopez Fernandez MF, Campos M, Justica B, Berges C, Navarro JL, Diaz Cremades JM, Kasper CK, Dent JA, Ruggeri ZM, Zimmerman TS: The heterogeneity of type IIA von Willebrand's disease: Studies with protease inhibitors. Blood 68: 1207–1212, 1986. 60 Zimmerman TS, Dent JA, Ruggeri ZM, Nannini LH: Subunit composition of plasma von Willebrand factor. Cleavage is present in normal individuals, increased in IIA and IIB von Willebrand disease, but minimal in variants with aberrant structure of individual oligomers ( types IIC, II D, and IIE). J Clin Invest 77: 947–951, 1986. 61 Ruggeri ZM, Pareti FI, Mannucci PM, Ciavarella N, Zimmerman TS: Heightened interaction between platelets and factor VIII/von Willebrand factor in a new subtype of von Willebrand's disease. N Engl J Med 302: 1047–1051, 1980. 62 Holmberg L, Nilsson IM, Borge L, Gunnarsson M, Sjorin E: Platelet aggregation induced by 1-desamino-8-D-arginine vasopressin (DDAVP) in Type IIB von Willebrand's disease. N Engl J Med 309: 816–821, 1983. 63 Federici A, Mannucci PM, Bader R, Lombardi R, Lattuada A: Heterogeneity in type IIB von Willebrand disease: two unrelated cases with no family history and mild abnormalities of ristocetin-induced interaction between von Willebrand factor and platelets. Am J Hematol 23: 381–390, 1986. 64 Gralnick HR, Williams SB, McKeown LP, Rick ME: von Willebrand's disease with spontaneous platelet aggregation induced by abnormal plasma von Willebrand factor. J Clin Invest 76: 1522–1529, 1985. 65 Ruggeri ZM, Nilsson IM, Lombardi R, Holmberg L, Zimmerman TS: Aberrant multimeric structure of von Willebrand factor in a new variant of von Willebrand's disease (type IIC). J Clin Invest 70: 1124–1127, 1982. 66 Mannucci PM, Lombardi R, Pareti FI, Solinas S, Mazzucconi MG, Mariani G: A variant of von Willebrand's disease characterized by recessive inheritance and missing triplet structure of von Willebrand factor multimers. Blood 62: 1000–1005, 1983. 67 Battle J, Lopez Fernandez MF, Lasierra J, Fernandez Villamor A, Lopez Berges C, Lopez Borrasca A, Ruggeri ZM, Zimmerman TS: von Willebrand disease type IIC with different abnormalities of von Willebrand factor in the same sibship. Am J Hematol 21: 177, 1986. 68 Batlle J, Lopez Fernandez MF, Fernandez Villamor A, Lopez Berges C, Zimmerman TS: Multimeric pattern discrepancy between platelet and plasma von Willebrand factor in type IIC von Willebrand disease. Am J Hematol 2: 87–88, 1986. 69 Mazurier C, Mannucci PM, Parquet Gernez A, Goudemand M, Meyer D: Investigation of a case of subtype IIC von Willebrand disease: Characterization of the variability of this subtype. Am J Hematol 22: 301–311, 1986. 70 Kinoshita S, Harrison J, Lazerson J, Abildgaard CF: A new variant of dominant type II von Willebrand's disease with aberrant multimeric pattern of factor VIII-related antigen (type IID). Blood 63: 1369–1371, 1984. 71 Hill FGH, Enayat MS, George AJ: Investigation of a kindred with a new autosomal dominantly inherited variant type von Willebrand's disease (possible type IID). J Clin Pathol 38: 665–670, 1985. 72 Mannucci PM, Lombardi R, Federici AB, Dent JA, Zimmerman TS, Ruggeri ZM: A new variant of type II von Willebrand disease with aberrant multimeric structure of plasma but not platelet von Willebrand factor (type IIF). Blood 68: 269–274, 1986. 73 Gralnick HR, Williams SB, McKeown LP, Maisonneuve P, Jenneau C, Sultan Y: A variant of Type II von Willebrand disease with an abnormal triplet structure and discordant effects of protease inhibitors on plasma and platelet von Willebrand factor structure. Am J Hematol 24: 259–266, 1987. 74 Mannucci PM, Lombardi R, Lattuada A, Muleo G, Federici AB: High-resolution multimeric analysis identifies a new variant of type II von Willebrand's disease (type II H) inherited in an autosomal recessive manner. Ric Clin Lab XVI: 237a, 1986. 75 Howard MA, Salem HH, Thomas KB, Hau L, Perkin J, Coghlan M, Firkin BG: Variant von Willebrand's disease type B-revisited. Blood 60: 1420–1428, 1982. 76 Ruggeri ZM, Mannucci PM, Lombardi R, Federici AB, Zimmerman TS: Multimeric composition of factor VIII/von Willebrand factor following administration of DDAVP: Implications for pathophysiology and therapy of von Willebrand's disease subtypes. Blood 59: 1272–1278, 1982. 77 Hanna WT, Slywka J, Dent J, Ruggeri ZM, Zimmerman TS: Case Report: 1-Deamino-8-D-Arginine vasopressin and cryoprecipitate in variant von Willebrand disease. Am J Hematol 20: 169–173, 1985. 78 Gralnick HR, Williams SB, McKeown P, Rick ME, Maisonneuve P, Jenneau C, Sultan Y: DDAVP in type IIa von Willebrand's disease. Blood 67: 465–468, 1986. 79 Weiss HJ, Meyer D, Rabinowitz R, Pietu G, Girma JP, Vicic WJ, Rogers J: Pseudo-von Willebrand's disease. An intrinsic platelet defect with aggregation by unmodified human factor VIII/von Willebrand factor and enhanced adsorption of its high-molecular-weight multimers. N Engl J Med 306: 326–333, 1982. 80 Miller JL, Castella A: Platelet-type von Willebrand's disease: characterization of a new bleeding disorder. Blood 60: 790–794, 1982. 81 Miller JL, Kupinski JM, Castella A, Ruggeri ZM: von Willebrand factor binds to platelets and induces aggregation in platelet-type but not type IIB von Willebrand disease. J Clin Invest 72: 1532–1542, 1983. 82 Ruggeri ZM, Lombardi R, Gatti L, Bader R, Valsecchi C, Zimmerman TS: Type IIB von Willebrand's disease: Differential clearance of endogenous versus transfused large multimer von Willebrand factor. Blood 60: 1453–1456, 1982. 83 Sakariassen KS, Cattaneo M, vd Berg A, Ruggeri ZM, Mannucci PM, Sixma JJ: DDAVP enhances platelet adherence and platelet aggregate growth on human artery subendothelium. Blood 64: 229–236, 1984. 84 Miller JL, Boselli BD, Kupinski JM: In vivo interaction of von Willebrand factor with platelets following cryoprecipitate transfusion in platelet-type von Willebrand's disease. Blood 63: 226–230, 1984. 85 Zimmerman TS, Abildgaard CF, Meyer D: The factor VIII abnormality in severe von Willebrand's disease. N Engl J Med 301: 1307–1310, 1979. 86 Ruggeri ZM, Mannucci PM, Jeffcoate SL, Ingram GIC: Immunoradiometric assay of Factor VIII related antigen, with observations in 32 patients with von Willebrand's disease. Br J Haematol 33: 221–232, 1976. 87 Shelton-Inloes BB, Chehab FF, Mannucci PM, Federici AB, Sadler JE: Gene deletions correlate with the development of alloantibodies in von Willebrand disease. J Clin Invest 79: 1459–1465, 1987. 88 Ngo K-Y, Lynch DC, Gitschier J, Ciavarella N, Ruggeri ZM, Zimmerman TS: Gene deletion in four patients from the same kindred with severe von Willebrand disease and anti-von Willebrand factor antibodies. Blood 68: 339a, 1986. 89 Mannucci PM, Meyer D, Ruggeri ZM, Koutts J, Ciavarella N, Lavergne JM: Precipitating antibodies in von Willebrand's disease. Nature 262: 141–142, 1976. 90 Ruggeri ZM, Ciavarella N, Mannucci PM, Molinari A, Dammacco F, Lavergne JM, Meyer D: Familial incidence of precipitating antibodies in von Willebrand's disease: A study of four cases. J Lab Clin Med 94: 60–75, 1979. 91 Mannucci PM, Ruggeri ZM, Ciavarella N, Kazatchkine MD, Mowbray JF: Precipitating antibodies to factor VIII/von Willebrand factor in von Willebrand's disease: effects on replacement therapy. Blood 57: 25–31, 1981. 92 Budde U, Schaefer G, Mueller N, Egli H, Dent J, Ruggeri Z, Zimmerman T: Acquired von Willebrand's disease in the myeloproliferative syndrome. Blood 64: 981–985, 1984. 93 Gill JC, Wilson AD, Endres-Brooks J, Montgomery RR: Loss of the largest von Willebrand factor multimers from the plasma of patients with congenital cardiac defects. Blood 67: 758–761, 1986. 94 Mohri H: Acquired von Willebrand disease and storage pool disease in chronic myelocytic leukemia. Am J Hematol 22: 391–401, 1986. 95 Moake JL, Rudy CK, Troll JH, Weinstein MJ, Colannino NM, Azocar J, Seder RH, Hong SL, Deykin D: Unusually large plasma factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med 307: 1432–1435, 1982. 96 Fuster V, Bowie EJW, Lewis JC, Fass DN, Owen CA Jr., Brown AL Jr.: Resistance to arteriosclerosis in pigs with von Willebrand's disease. J Clin Invest 61: 722–730, 1978. 97 Bowie EJW, Fuster V, Fass DN, Owen CA Jr.: The role of Willebrand factor in platelet-blood vessel interaction, including a discussion of resistance to atherosclerosis in pigs with von Willebrand's disease. Philos Trans R Soc Lond [Biol] 294: 267–279, 1981. 98 Nichols TC, Bellinger DA, Johnson TA, Lamb MA, Griggs TR: von Willebrand's disease prevents occlusive thrombosis in stenosed and injured porcine coronary arteries. Circ Res 59: 15–26, 1986. Citing Literature Volume1, Issue41987Pages 353-362 ReferencesRelatedInformation