Inherited disorders of platelet α-granules

Platelets

Volumn 8, Issue 4, 1997, Pages 197-210

  Hayward, Catherine P M ^a,b  

^a MCMASTER UNIVERSITY   (Canada)

^b HAMILTON HEALTH SCIENCES   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

PADGEM PROTEIN; PLASMA PROTEIN; PLASMINOGEN ACTIVATOR INHIBITOR 1;

AFIBRINOGENEMIA; ARTICLE; BLEEDING DISORDER; BLOOD CLOTTING FACTOR 5 DEFICIENCY; CELL GRANULE; CLINICAL FEATURE; CONTROLLED STUDY; GLANZMANN DISEASE; HEMOSTASIS; HUMAN; HUMAN CELL; INHERITANCE; LABORATORY TEST; MEGAKARYOCYTE; PRIORITY JOURNAL; PROTEIN DEFECT; THROMBOCYTE DISORDER; THROMBOCYTOPENIA; VON WILLEBRAND DISEASE;

EID: 0030747037     PISSN: 09537104     EISSN: None     Source Type: Journal    
DOI: 10.1080/09537109777249     Document Type: Article

References (106)

1. Harrison P, Savidge GF, Cramer EM. The origin and physiological relevance of α-granule adhesive proteins. Br J Haematol 1990: 74: 125-30.
2. Harrison P, Cramer EM. Platelet α-granules. Blood Rev 1993: 7: 52-62.
3. Cramer EM, Meyer D, le Menn R, Breton Gorius J. Eccentric localization of von Willebrand factor in an internal structure of platelet α-granule resembling that of Weibel-Palade bodies. Blood 1985; 66: 710-3.
4. Cramer EM, Caen JP, Drouet L, Breton-Gorius J. Absence of tubular structures and immunolabeling for von Willebrand factor in the platelet α-granules from porcine von Willebrand disease. Blood 1986; 68: 774-8.
5. Cramer EM, Beesley J, Breton-Gorius J, Martin JF. Ulstrslruetural demonstration of tubular inclusions coinciding with von Willebrand factor in pig megakaryocytes. Blood 1988; 71: 1533-8.
6. Wilbourn B, Harrison P, Lawrie A, Savariau E, Savidge G, Cramer EM. Porcine platelets contain an increased quantity of ultra-high molecular weight von Willebrand factor and numerous alpha-granular tubular structures. Br J Haematol 1993; 83: 608-15.
7. Roussi J, Drouet L, Sigman J, Vaiman M, Pignaud G. Bonneau M, Masse J-M, Cramer EM. Absence of incorporation of plasma von Willebrand factor into porcine platelet α-granules. Br J Haematol 1995; 90: 661-8.
8. Hayward CPM, Bainton DF, Smith JW, Horsewood P, Stead RH, Podor TJ, Warkentin TE, Kelton JG. Multimerin is found in the α-granutes of resting platelets and is synthesized by a megakaryocytic cell line. J Clin Invest 1993; 91: 2630-9.
9. Hayward CPM, Furmaniak-Kazmierczak E, Cieutat A-M. Moore JC, Bainton DF, Nesheim ME, Kelton JG, Côté G. Factor V is complexed with multimerin in resting platelet lysates and colocalizes with multimerin in platelet α-granules. J Biol Chem 1995; 270: 19217-24.
10. Hsu-Lin S, Berman CL, Furie BC, August D, Furie B. A platelet membrane protein expressed during platelet activation and secretion. J Biol Chem 1984; 259: 9121-6.
11. McEver RP, Martin MN. A monoclonal antibody to a membrane glycoprotein binds only to activated platelets. J Biol Chem 1984; 259: 9799-804.
12. Stenberg PE, McEver RP, Shuman MA, Jacques YV, Bainton DF. A platelet alpha-granule membrane protein (GMP-140) is expressed on the plasma membrane after activation. J Cell Biol 1985; 101: 880-6.
13. Berger G, Masse J-M, Cramer EM. Alpha-granule membrane mirrors the platelet plasma membrane and contains glycoproteins Ib, IX and V. Blood 1996; 87: 1385-95.
14. Cramer EM, Savidge GF, Vainchenker W, Berndt MC, Pidard D, Caen JP, Masse J-M, Breton-Borius J. Alpha-granule pool of glycoprotein IIb-IIIa in normal and pathologic platelets and megakaryocytes. Blood 1990; 75: 1220-7.
15. Raccuglia G. Gray platelet syndrome. A variety of qualitative platelet disorder. Am J Med 1971; 51: 818-28.
16. White JG. Ultrastructural studies of the gray platelet syndrome. Am J Pathol 1979; 95: 445-53.
17. Gerrard JM, Phillips DR, Rao GHR, Plow EF, Walz DA, Ross R, Harker LA, White JG. Biochemical studies of two patients with the gray platelet syndrome. Selective deficiency of platelet alpha granules. J Clin Invest 1980; 66: 102-9.
18. Levy-Toledano S, Caen JP, Breton-Gorius J, Rendu F, Cywiner-Golenzer C, Dupuy E, Legrand Y, Maclouf J. Gray platelet syndrome: ́-granule deficiency. Its influence on platelet function. J Lab Clin Med 1981; 98: 831-48.
19. Breton-Gorius J, Vainchenker W, Nurden A, Levy-Toledano S, Caen J. Detective alpha-granule production in megakaryocytes from gray platelet syndrome: Ultrastructural studies of bone marrow cells and megakaryocytes growing in culture of blood precursors. Am J Pathol 1981; 102: 10-19.
20. Nurden AT, Kunicki TJ, Dupuis D, Soria C, Caen JP. Specific-protein and glycoprotein deficiencies in platelets isolated from two patients with the gray platelet syndrome. Blood 1982; 59: 709-18.
21. Berndt MC, Castaldi PA, Gordon S, Halley H, McPherson VJ. Morphological and biochemical confirmation of gray platelet syndrome in two siblings. Aust NZ J Med 1983; 13: 387-90.
22. Cramer EM, Vainchecker W, Vinci G, Guichard J, Breton-Gorius J. Gray platelet syndrome: Immunoelectron microscopic localization of fibrinogen and von Willebrand factor in platelets and megakaryocytes. Blood 1985; 66: 1309-16.
23. Mori K, Suzuki S, Sugai K. Electron microscopic and functional studies on platelets in gray platelet syndrome. Tohoku J Exp Med 1984; 143: 261-87.
24. Köhler M, Hellstern P, Morgenstern E, Mueller-Eckhardt C, Berberich R, Meiser RJ, Scheffler P, Wenzel E. Gray platelet syndrome: selective α-granule deficiency and thrombocytopenia due to increased platelet turnover. Blut 1985; 50: 331-40.
25. Greenberg-Sepersky SM, Simons ER, White JG. Studies of platelets from patients with the gray platelet syndrome. Br J Haematol 1985; 59: 603-9.
26. Rosa J-P, George JN, Bainton DF, Nurden AT, Caen JP, McEver RP. Gray platelet syndrome. Demonstration of alpha granule membranes that can fuse with the cell surface. J Clin Invest 1987; 80: 1138-46.
27. Studies on granule-bound substances in 18 patients including variants deficient in α-granules, platelet factor 4, β-thromboglobulin and platelet-derived growth factor. Blood 1979; 54: 1296-319.
28. Weiss HJ, Chervenick PA, Zalusky R, Factor A. A familial defect in platelet function associated with impaired release of adenine diphosphate. New Engl J Med 1969; 281: 1264-70.
29. Weiss HJ, Lages B, Vicic W, Tsung LY, White JG. Heterogeneous abnormalities of platelet dense granule ultrastucture in 20 patients with congenital storage pool deficiency. Br J Haematol 1993; 83: 282-95.
30. Hayward CPM, Rivard GE, Kane WH, Drouin J, Zheng S, Moore JC, Kelton JG. An autosomal dominant, qualitative platelet disorder associated with multimerin deficiency, abnormalities in platelet factor V, thrombospondin, von Willebrand factor, and fibrinogen, and an epinephrine aggregation defect. Blood 1996; 87: 4967-78.
31. Hayward CPM, Cramer EM, Kane WH, Zheng S, Bouchard M, Massé J-M, Rivard GE. Studies of a second family with the Quebec platelet disorder: evidence that the degradation of the α-granule membrane and its soluble contents are not secondary to a defect in targeting proteins to α-granules. Blood 1997; 88: 1243-53.
32. Tracy PB, Giles AR, Mann KG, Eide LL, Hoogendoorn H, Rivard GE. Factor V (Quebec): a bleeding diathesis associated with a qualitative platelet factor V deficiency. J Clin Invest 1984; 74: 1221-8.
33. Janeway C, Rivard GE, Tracy PB, Mann KG. Factor V Quebec revisited. Blood 1996; 87: 3571-8.
34. Breton-Gorius J, Favier R, Guichard J, Cherif D, Berger R, Debili N, Vainchenker W, Douay L. A new congenital dysmegakaryopoietic thrombocytopenia (Paris-Trousseau) associated with giant platelet α-granules and chromosome 11 deletion at 11q23. Blood 1995; 85: 1805-14.
35. Gangarossa S, Schiliró G, Mattina T, Scardilli S, Mollica F, Cavallari V. Dysmegakeryopoietic thrombocytopenia in patients with distal chromosome 11q deletion. Blood 1996; 87: 4915-6.
36. Kane WH, Davie EW. Blood coagulation factor V and VIII: structural and functional similarities and their relationship to hemorrhagic and thrombotic disorders. Blood 1988; 71: 539-55.
37. Miletich JP, Majerus DW, Majerus PW. Patients with congenital Factor V deficiency have decreased Factor Xa binding sites on their platelets. J Clin Invest 1978; 62: 824-31.
38. Soria J, Soria C, Borg JY, Mirshahi M, Piguet H, Tron P, Fessard C, Caen JP. Platelet aggregation occurs in congenital afibrinogenemia despite the absence of fibrinogen of its fragments in plasma and platelets, as demonstrated by immunoenzymology. Br J Haematol 1985; 60: 503-14.
39. Cattaneo M, Kinlough-Rathbone RL, Lecchi A, Bevilacqua C, Packham MA, Mustard JF. Fibrinogen independent aggregation and disaggregation of human platelets: studies in two afibrinogenemic patients. Blood 1987; 70: 221-6.
40. Rachman RL, Marcus AJ. Immunological studies of proteins associated with subcellular fractions of thrombasthenic and afibrinogenemic platelets. Br J Haematol 1968; 15: 181-9.
41. Al-Mondhiry H, Ehmann WC. Congenital afibrinogenemia. Am J Hematol 1994; 46: 343-7.
42. Disdier M, Legrand C, Bouillot C, Duberhard V, Pidard D, Nurden AT. Quantitation of platelet Fg and thrombospondin in Glanzmann's thrombasthenia by electroimmunoassay. Thromb Res 1989; 53: 521-33.
43. Belloc F, Heilmann E, Combrie R, Boisseau MR, Nurden AT. Protein synthesis and storage of fibrinogen in Glanzmann's thrombasthenia. Biochem Biophys Acta 1987; 925: 218-25.
44. Weiss HJ, Pietu G, Rabinowitz R, Girma J-P, 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 1983; 101: 411-25.
45. 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 1985; 66: 796-802.
46. Gralnick HR, Rick ME, McKeown LP, Williams SB, Parker RI, Maisonneuve P, Jeanneau C, Sultan Y. Platelet von Willebrand factor: an important determinant of the bleeding time in type I von Willebrand's disease. Blood 1986; 68: 58-61.
47. Wilbourn B, Harrison P, Lawrie A, Savariau E, Savidge G, Cramer EM. Porcine platelets contain an increased quantity of ultra-high molecular weight von Willebrand factor and numerous alpha-granular tubular structures. Br J Haematol 1993; 83: 608-15.
48. Roussi J, Drouet L, Vaiman M, Pignaud G, Bonneau M, Masse J-M, Cramer EM. Absence of incorporation of plasma von Willebrand factor into porcine platelet α-granules. Br J Haematol 1995; 90: 661-8.
49. Fay WP, Shapiro AD, Shih JL, Schleef RR, Ginsberg D. Brief report: complete deficiency of plasminogen-activator inhibitor type 1 due to a frame-shift mutation. New Engl J Med 1992; 327: 1729-33.
50. Jantunen E, Hänninen A, Naukkarinen A, Vornancn M, Lahtinen R. Gray platelet syndrome with splenomegaly and signs of extramedullary hematopoiesis: a case report with review of the literature. Am J Hematol 1994; 46: 218-24.
51. Caen JP, Deschamps JF, Bodevin E, Bryckaert MC, Dupuy E, Wasteson A. Megakaryocytes and myelofibrosis in gray platelet syndrome. Nouv Rev Fr Haematol 1987; 29: 109-14.
52. Gootenberg JE, Buchanan GR, Holtkamp CA, Casey CS. Severe hemorrhage in a patient with gray platelet syndrome. J Pediatrics 1986; 109: 1017-9.
53. Berrebi A, Klepfish A, Varon D, Shtalrid M, Vorst E, Nir E, Lavac J. Gray platelet syndrome in the elderly. Am J Hematol 1988; 28: 270-2.
54. Facon T, Goudemand J, Caron C, Zandecki M, Estienne M-H, Fenaux P, Cosson A. Simultaneous occurrence of grey platelet syndrome and idiopathic pulmonary fibrosis: a role for abnormal megakaryocytes in the pathogenesis of pulmonary fibrosis? Br J Haematol 1990; 74: 542-3.
55. Pfueller SL, Howard MA, White JG, Menon C, Berry EW. Shortening of the bleeding time by 1-deamino-8-arginine vasopressin (DDAVP) in the absence of platelet von Willebrand factor in gray platelet syndrome. Thromb Haemost 1987; 58: 1060-3.
56. Köhler M. Treatment of gray platelet syndrome. Thromb Haemost 1988; 60: 123.
57. Cockbill SR, Burmester HBC, Heptinstall S. Pseudo grey platelet syndrome - grey platelets due to degranulation in blood collected into EDTA. Eur J Haematol 1988; 41: 326-33.
58. Srivastava PC, Powling MJ, Nokes TJ, Patrick AD, Dawes J, Hardisty RM. Grey platelet syndrome: studies on platelet alpha-granules, lysosomes and defective response to thrombin. Br J Haematol 1987; 65: 441-6.
59. Lages B, Sussmann II, Levine SP, Coletti D, Weiss HJ. Platelet alpha granule deficiency associated with decreased P-selectin and selective impairment of thrombin-induced activation in a new patient with gray platelet syndrome (α-storage pool deficiency). J Lab Clin Med 1997; 129: 364-75.
60. Enouf J, Lebret M, Bredoux R, Levy-Toledano S, Caen JP. Abnormal calcium transport into microsomes of grey platelet syndrome. Br J Haematol 1987; 65: 437-50.
61. Morgenstern E, Patscheke H, Mathieu G. The origin of the membrane convolute in degranulating platelets. A comparative study of normal and 'gray' platelets. Blut 1990; 60: 15-22.
62. Baruch D, Lindhout T, Dupuy E, Caen J-P. Thrombin-induced platelet factor Va formation in patients with a gray platelet syndrome. Thromb Haemostas 1987; 58: 768-71.
63. Vermylen C, Vermylen J, Hoet B, Scheiff JM, Ninane J, Cornu G. Grey platelet syndrome: evidence for alpha-granule localization of the platelet plasminogen activator inhibitor-1 pool. Pediatr Hematol Oncol 1991; 8: 111-20.
64. Pfueller SL, David R. Platelet-associated immunoglobulins G. A. and M are secreted during platelet activation: normal levels but defective secretion in grey platelet syndrome. Br J Haematol 1988; 68: 235-41.
65. Boukerche H, Ruchaud-Spragano M-H, Rouen C, Brochier J, Kaplan C, McGregor JL. A monoclonal antibody directed against a granule membrane glycoprotein (GMP-140/PADGEM. P-selectin, CD62P) inhibits ristocetin-induced platelet aggregation. Br J Haematol 1996; 92: 44-51.
66. Mazurov AV, Vinogradov DV, Khaspekova SG, Krushinsky AV, Gerdeva LV, Vasiliev SA. Deficiency of P-selectin in a patient with grey platelet syndrome. Eur J Haematol 1996; 57: 38-41.
67. Gebrane-Younès, Cramer EM, Orcel L, Caen JP. Gray platelet syndrome. Dissociation between abnormal sorting in megakaryocyte α-granules and normal sorting in Weibel-Palade bodies of endothelial cells. J Clin Invest 1993; 92: 3023-8.
68. Jackson CW, Hutson NK, Steward SA, Saito N, Cramer EM. Platelets of the Wistar Furth rat have reduced levels of alpha-granule proteins. An animal model resembling gray platelet syndrome. J Clin Invest 1991; 87: 1985-91.
69. Pestina TI, Jackson CW, Stenherg PE. Abnormal subcellular distribution of myosin and talin in Wistar Furth rat platelets. Blood 1995; 85: 2436-46.
70. Lages B, Shattil S, Bainton DF, Weiss HJ. Decreased content and surface expression of alpha-granule membrane protein GMP-140 in one of two types of platelet αδ storage pool deficiency. J Clin Invest 1991; 87: 919-29.
71. Weiss HJ, Lages B. Studies of thromboxane B2, platelet factor 4. and fibrinopeptide A in bleeding time blood of patients deficient in von Willebrand factor, platelet glycoproteins Ib, IIb-IIIa, and storage granules. Blood 1993; 82: 481-90.
72. Weiss HJ, Lages B. The response of platelets to epinephrine in storage pool deficiency - evidence pertaining to the role of ADP in mediating primary and secondary aggregation. Blood 1988; 72: 1717-25.
73. 2 collagen, and epinephrine in 12 patients with storage pool deficiency. Blood 1981; 58: 27-33.
74. Weiss HJ, Turitto VT, Baumgartner HR. Platelet adhesion and thrombus formation on subendothelium in platelets deficient in glycoproteins IIb-IIIa, Ib, and storage granules. Blood 1986; 67: 322-30.
75. White JG. Inherited abnormalities of the platelet membrane and secretory granules. Hum Pathol 1987; 18: 123-39.
76. Vicie WJ, Weiss HJ. Evidence that platelet α-granules are a major determinant of platelet density: studies in storage pool deficiency. Thromb Haemostas 1983; 50: 878-80.
77. Lages B, Dangelmaier CA, Holmsen H, Weiss HJ. Specific-correction of impaired acid hydrolase secretion in storage pool-deficient platelets by adenosine diphosphate. J Clin Invest 1988; 81: 1865-72.
78. Weiss HJ, Lages B. Platelet prothrombinase activity and intracellular calcium responses in platelets with glycoprotein IIb-IIIa deficiency, granule defects, or impaired platelet procoagulant activity - a comparison with Scott syndrome. Blood 1997; 89: 1599-611.
79. Swank RT, Jiang SY, Reddington M, Conway J, Stephenson D, McGarry MP, Novak EK. Inherited abnormalities in platelet organelles and platelet formation and associated altered expression of low molecular weight guanosine triphosphate-binding proteins in the mouse pigment mutant gunmetal. Bood 1993; 81: 2626-35.
80. Novak EK, Reddington M, Zhen L, Stenberg PE, Jackson CW, McGarry MP, Swank RT. Inherited thrombocytopenia caused by reduced platelet production in mice with the gunmetal pigment gene mutation. Blood 1995; 85: 1781-9.
81. Hayward CPM. Multimerin: a unique platelet and endothelial cell protein. From discovery to function to abnormalities in disease. A bench to bedside chronology. Clin Invest Med 1997; 20: 176-87.
82. Hayward CPM, Smith JW, Horsewood P, Warkentin TE, Kelton JG. p-155, a multimeric platelet protein that is expressed on activated platelets. J Biol Chem 1991; 266: 7114-20.
83. Hayward CPM, Warkentin TE, Horsewood P, Kelton JG. Multimerin: a series of large, disulfide-linked multimeric proteins within platelets. Blood 1991; 77: 2556-60.
84. Hayward CPM, Hassell JA, Denomme GA, Rachubinski RA, Brown C, Kelton JG. The cDNA sequence of human endothelial cell multimerin: A unique protein with RGDS, coiled-coil, and EGF-like domains and a carboxyl-terminus similar to the globular domain of complement Clq and collagens type VIII and X. J Biol Chem 1995; 270: 18242-51.
85. Hayward CPM, Welch B, Bouchard M, Zheng S, Rivard GE. Fibrinogen degradation products in patients with the Quebec platelet disorder. Br J Haematol 1977; 97: 497-503.
86. Gangarossa S, Mattina T, Romano V, Milana G, Mollica F, Schiliró G. Micromegakaryocytes in a patient with partial deletion of the long arm of chromosome 11 [del(11)q24.2qter)] and chronic thrombocytopenic purpura. Am J Med Genet 1996; 62: 120-3.
87. Penny LA, Dell'Aquila M, Jones MC, Bergoffen J, Cunniff C, Fryns J-P, Grace E, Graham JM Jr. Kouseff B, Mattina T, Syme J, Voullaire L, Zelante L, Zenger L, Zenger Haim J, Jones OW. Evans GA. Clinical and molecular characterization of patients with distal 11q deletions. Am J Hum Genet 1995; 56: 676-83.
88. Bowie EJ, Solberg LA Jr, Fass DN, Johnson CM, Stewart ML, Zoecklein LJ. Transplantation of normal bone marrow into a pig with severe vWd. J Clin Invest 1986; 78: 26-30.
89. Federici AB, DeGroot PG, Moia M, Ijsseldijk MJW, Sixma JJ, Mannucci PM. Type I von Willebrand disease, subtype 'platelet low': decreased platelet adhesion can be explained by low synthesis of von Willebrand factor in endothelial cells. Br J Haematol 1993; 83: 88-93.
90. Ewenstein BM, Inbal A, Pober JS, Handin RI. Molecular studies of von Willebrand disease: reduced von Willebrand factor biosynthesis, storage and release in endothelial cells derived from patients with type I von Willebrand disease. Blood 1990; 75: 1466-72.
91. Roussi J, Drouet L, Sigman J, Vaiman M, Pignaud G, Bonneau M, Masse J-M, Cramer EM. Absence of incorporation of plasma von Willebrand factor into porcine platelet α-granules. Br J Haemat 1995; 90: 661-8.
92. Mannucci PM. Platelet von Willebrand factor in inherited and acquired bleeding disorders. Proc Natl Acad Sci 1995; 92: 2428-32.
93. Nichols TC, Samama CM, Bellinger DA, Roussi J, Reddick RL, Bonneau M, Read MS, Bailliart O, Koch GG, Vaiman M, Sigman JL, Pignaud GA, Brinkhous KM, Griggs TR, Drouet L. Function of von Willebrand factor after crossed bone marrow transplantation between normal and von Willebrand disease pigs: effect on arterial thrombosis in chimeras. Proc Natl Acad Sci 1995; 92: 2455-9.
94. Chediak J, Ashenhurst JB, Garlick I, Desser RK. Successful management of bleeding in a patient with factor V inhibitor by platelet transfusions. Blood 1980; 56: 835-41.
95. Borchgrevink CF, Owren PA. The hemostatic effect of normal platelets in hemophilia and factor V deficiency. The importance of clotting factors adsorbed on platelets for normal hemostasis. Ada Med Scand 1961; 170: 375-83.
96. Nesheim ME, Nichols WL, Cole WL, Cole TL, Houston JG, Schenk RB, Mann KG, Bowie EJW. Isolation and study of an acquired inhibitor of human coagulation factor V. J Clin Invest 1986; 77: 405-15.
97. Harrison P, Wilbourn B, Cramer E, Faint R, Mackie JJ, Bhattacharya S, Lahiri A, Tenza D, Machin SJ, Savidge SG. The influence of therapeutic blocking of GP IIb/IIIa on platelet α-granular fibrinogen. Br J Haematol 1992; 82: 721-8.
98. Harrison P, Wilbourn BR, Debili N, Vainchecker W, Breton-Gorius J, Lawrie AS, Masse J-M, Savidge GF, Cramer EM. Uptake of plasma fibrinogen into the α-granules of human megakaryocytes and platelets. J Clin Invest 1989; 84: 1320-4.
99. Fressinaud E, Federici AB, Castaman G, Rothschild C, Rodeghiero F, Baumgartner HR, Mannucci PM, Meyer D. The role of platelet von Willebrand factor in platelet adhesion and thrombus formation: a study of 34 patients with various subtypes of type I von Willebrand disease. Br J Haematol 1994; 86: 327-32.
100. Castillo R, Escolar G, Monteagudo Y, Ordinas A, Garrido M, Moia M, Federici AB, Mannucci PM. Role for platelet von Willebrand factor in supporting platelet-vessel wall interactions in von Willebrand disease. Am J Hematol 1989; 31: 153-8.
101. D'Alessio P, Zwaginga JJ, deBoer HC, Federici AB, Rodeghiero F, Castaman G, Mariani G, Mannucci PM, deGroot PG, Sixma JJ. Platelet adhesion to collagen in subtypes of type I von Willebrand's disease is dependent on platelet von Willebrand factor. Thromb Haemost 1990; 64: 227-31.
102. Fressinaud E, Baruch D, Rothschild C, Baumgartner HS, Meyer D. Platelet von Willebrand factor: evidence for its involvement in platelet adhesion to collagen. Blood 1987; 70: 1214-7.
103. Lattuada A, Varanukulsak P, Castaman GC, Mannucci PM. The response of plasma von Willebrand factor to desmopressin (DDAVP) is related to the platelet levels of von Willebrand factor. Thromb Res 1992; 67: 467-71.
104. Eller T, Brauer P, Albert J, Mayer J, Keller F. Significance and quantitative analysis of von Willebrand factor in human platelets. Thromb Res 1992; 65: 631-40.
105. Gralnick HR, Rick ME, McKeown LP, Williams SB, Parker RI, Maisonneuve P, Jenneau C, Sultan Y. Platelet von Willebrand factor: an important determinant of the bleeding time in type I von Willebrand's disease. Blood 1986; 68: 58-61.
106. McKeown LP, Williams SB, Shafer B, Murray N, Gralnick HR. Alpha granule proteins in type I von Willebrand's disease. Am J Hematol 1993; 42: 158-61.