Hemostatic Alterations in Sickle Cell Disease: Relationships to Disease Pathophysiology

References

• Bunn H F. Pathogenesis and treatment of sickle cell disease. New Engl J Med 1997; 337: 762–769
   PubMed Web of Science ®Google Scholar
• Stockman J A, Nigro M A, Mishkin M M, Oski F A. Occlusion of large cerebral vessels in sickle cell anemia. New Engl J Med 1972; 287: 846–849
   PubMed Web of Science ®Google Scholar
• Merkel K H, Ginsberg P L, Parker J C, Donovan Post M J. Cerebrovascular disease in sickle cell anemia: a clinical, pathological, and radiological correlation. Stroke 1978; 9: 45–52
   PubMed Web of Science ®Google Scholar
• Ohene-Frempong K, Weiner S J, Sleeper L A. Cerebrovascular accidents in sickle cell disease: rates and risk factors. Blood 1998; 91: 288–294
   PubMed Web of Science ®Google Scholar
• Oppenheimer E H, Esterly J R. Pulmonary changes in sickle cell disease. Am Rev Respir Dis 1971; 103: 858
   PubMedGoogle Scholar
• Haupt H M, Moore G W, Bauer T W, Hutchins G M. The lung in sickle cell disease. Chest 1982; 81: 332–337
   PubMedGoogle Scholar
• Francis R B, Johnson C S. Vascular occlusion in sickle cell disease. Current concepts and unanswered questions. Blood 1991; 77: 1405–1414
   PubMed Web of Science ®Google Scholar
• Khademi M, Marquis J R. Renal angiography in sickle cell disease. Radiology 1973; 107: 41
   Google Scholar
• Klug P P, Kaye N, Jensen W N. Endothelial cell and vascular damage in the sickle cell disorders. Blood Cells 1982; 8: 175
   PubMedGoogle Scholar
• Serjeant G R. Sickle Cell Disease. Oxford University, New York 1985; 164
   Google Scholar
• Lutty G A, Goldberg M F. Ophthalmologic complications in sickle cell disease. Basic Principles and Clinical Practice, S H Embury, R P Hebbel, N Mohandas, M H Steinberg. Raven Press, New York 1994; 703–724
   Google Scholar
• Koshy M, Burd I, Wallace D, Moawad A, Baron J. Prophylactic red cell transfusions in pregnant patients with sickle cell disease. A randomized cooperative study. N Engl J Med 1988; 319: 1447
   PubMed Web of Science ®Google Scholar
• Zwaal R FA, Schroit A J. Pathophysiologic implications of membrane phospholipid asymmetry in blood cells: a review. Blood 1997; 89: 1121–1132
   PubMed Web of Science ®Google Scholar
• Chiu D, Lubin B, Shohet S B. Erythrocyte membrane lipid reorganization during the sickling process. Br J Haematol 1979; 41: 223
   PubMedGoogle Scholar
• Franck P FH, Bevers E M, Lubin B H, Comfurius P, Chiu D, Op den kamp J AF, Zwaal R FA, van Deenen L LM, Roelofsen B. Uncoupling of the membrane skeleton from the lipid bilayer. The cause of accelerated phospholipid flip-flop leading to an enhanced procoagulant activity of sickled cells. J Clin Invest 1985; 75: 183
   PubMed Web of Science ®Google Scholar
• Zwaal R FA, Bevers E M, Comfurius P, Rosing J, Tilly R HJ, Verhallen P FJ. Loss of membrane phospholipid asymmetry during activation of blood platelets and sickled red cells. Mechanism and physiologic significance. Mol Cell Biochem 1989; 91: 23
   PubMed Web of Science ®Google Scholar
• Tait J F, Gibson D. Measurement of membrane phospholipid asymmetry in normal and sickle erythrocytes by means of annexin V binding. J Lab Clin Med 1994; 123: 741
   PubMed Web of Science ®Google Scholar
• Kuypers F A, Lewis R A, Hua M, Scholt M A, Discher D, Ernst J D, Lubin B H. Detection of altered membrane phospholipid asymmetry in subpopulations of human red blood cells using fluorescently labeled annexin V. Blood 1996; 87: 1179
   PubMed Web of Science ®Google Scholar
• Wood B L, Gibson D F, Tait J F. Increased erythrocyte phosphatidylserine exposure in sickle cell disease: flow-cytometric measurement and clinical associations. Blood 1996; 88: 1873
   PubMed Web of Science ®Google Scholar
• Schroit A J, Zwaal R FA. Transbilayer movement of phospholipids in red cell and platelet membranes. Biochim Biophys Acta 1991; 1071: 313–329
   PubMed Web of Science ®Google Scholar
• Seigneuuret M, Devaux P F. Asymmetric distribution of spin-labeled phospholipids in the erythrocyte membrane: relation to shape changes. Proc Natl Acad Sci. USA 1984; 81: 3751
   Google Scholar
• Devaux P F, Zachowski A. Maintenance and consequences of membrane phospholipid asymmetry. Chem Phys Lipids 1994; 73: 107
   Web of Science ®Google Scholar
• Bitbol M, Devaux P F. Measurement of outward translocation of phospholipids across human erythrocyte membrane. Proc Natl Acad Sci USA 1988; 85: 6783
   PubMed Web of Science ®Google Scholar
• Connor J, Pak C H, Zwaal R FA, Schroit A J. Bidirectional transbilayer movement of phospholipid analogs in human red blood cells. J Biol Chem 1992; 267: 19412
   PubMed Web of Science ®Google Scholar
• Diaz C, Schroit A J. Role of translocases in the generation of phosphatidylserine asymmetry. J Membr Biol 1996; 151: 1
   PubMed Web of Science ®Google Scholar
• Zwaal R FA, Comfurius P, Bevers E M. Mechanism and function of changes in membrane phospholipid asymmetry in platelets and erythrocytes. Biochem Soc Trans 1993; 21: 248
   PubMed Web of Science ®Google Scholar
• De Jong K, Rossum L, Lubin B H, Styles L A, Kuypers F A. Phosphatidylserine externalization in subpopulations of sickle cells. Blood 1997; 90: 125a
   Google Scholar
• Allan D, Limbrick A R, Thomas P, Westerman M P. Release of spectrin-free spicules on reoxygenation of sickled erythrocytes. Nature (Lond) 1982; 295: 612
   PubMedGoogle Scholar
• Blumenfeld N, Zachowski A, Galacteros F, Beuzard Y, Devaux P F. Transmembrane mobility of phospholipids in sickle erythrocytes. Effect of deoxygenation on diffusion and asymmetry. Blood 1991; 77: 849
   PubMed Web of Science ®Google Scholar
• Liu S C, Yi S J, Mehta J R, Nichols P E, Ballas S K, Yacono P W, Golan D E, Palek J. Red cell membrane remodeling in sickle cell anemia: sequestration of membrane lipids and proteins in Heinz bodies. J Clin Invest 1995; 97: 29
   Google Scholar
• De Jong K, Geldwerth D, Kuypers F A. Oxidative damage does not alter membrane phospholipid asymmetry in human erythrocytes. Biochemistry 1997; 36: 6768
   PubMed Web of Science ®Google Scholar
• Brugnara C. Cation homeostasis. Sickle Cell Disease: Basic Principles and Clinical Practice, S H Embury, R P Hebbel, N Mohandas, M H Steinberg. Raven Press, New York 1994; 173–194
   Google Scholar
• Seligsohn U, Osterud B, Brown S F, Griffin J H, Rapaport S I. Activation of human factor VII in plasma and in purified systems. Roles of activated factor IX, Kallikrein and activated factor XII. J Clin Invest 1979; 64: 1056
   PubMed Web of Science ®Google Scholar
• Nemerson Y, Repke D. Tissue factor accelerates the activation of factor VII. Thromb Res 1985; 40: 351
   PubMed Web of Science ®Google Scholar
• Bach R, Gentry R, Nemerson Y. Factor VII binding to tissue factor in reconstituted phospholipid vesicles. Induction of cooperativity by phosphatidylserine. Biochemistry 1986; 25: 4007
   PubMed Web of Science ®Google Scholar
• Rosing J, van Rijn J L, Bevers E M, van Dieijen G, Comfurius P, Zwaal R FA. The role of activated human platelets in prothrombin and factor X activation. Blood 1985; 65: 319–332
   PubMed Web of Science ®Google Scholar
• Chiu D, Lubin B, Roelofsen B, van Deenen L LM. Sickled erythrocytes accelerate clotting in vitro: an effect of abnormal membrane lipid asymmetry. Blood 1981; 58: 398–402
   PubMed Web of Science ®Google Scholar
• Francis R B, Hebbel R P. Hemostasis in sickle cell disease. Basic Principles and Clinical Practice, S H Embury, R P Hebbel, N Mohandas, M H Steinberg. Raven Press, New York 1994; 299–310
   Google Scholar
• Leslie J, Langler D, Serjeant G R, Serjeant B E, Desai P, Gordon Y B. Coagulation changes during the steady state in homozygous sickle cell disease in Jamaica. Br J Haematol 1975; 30: 159–166
   PubMed Web of Science ®Google Scholar
• Green D, Scott J P. Is sickle cell crisis a thrombotic event?. Am J Hematol 1986; 23: 317–321
   PubMed Web of Science ®Google Scholar
• Devine D V, Kinney T R, Thomas P F, Rosse W F, Greenberg C S. Fragment D-Dimer levels: an objective marker of vasoocclusive crisis and other complications of sickle cell disease. Blood 1986; 68: 317–319
   Web of Science ®Google Scholar
• Francis R B. Elevated fibrin D-dimer fragment in sickle cell anemia: evidence for activation of coagulation during the steady state as well as in painful crisis. Haemostasis 1989; 19: 105–111
   PubMedGoogle Scholar
• Kurantsin-Mills J, Ofosu F A, Safa T K, Siegel R S, Lessin L S. Plasma factor VII and thrombin-antithrombin III levels indicate increased tissue factor activity in sickle cell patients. Br J Haematol 1992; 81: 539–544
   PubMed Web of Science ®Google Scholar
• Peters M, Plaat B E, ten Gate H, Wolters H J, Weening R S, Brandjes D P. Enhanced thrombin generation in children with sickle cell disease. Thrombos Haemostas 1994; 71: 169–172
   PubMed Web of Science ®Google Scholar
• Liesner R, Mackie I, Cookson J, McDonald S, Chitolie A, Donohoe S, Evans J, Hann I, Machin S. Prothrombofic changes in children with sickle cell disease: relationships to cerebrovascular disease and transfusion. Br J Haematol 1998; 103: 1037–1044
   PubMed Web of Science ®Google Scholar
• Westerman M P, Green D, Gilman-Sachs A, Beaman K, Freels S, Boggio L, Allen S, Zuckerman L, Schlegel R, Williamson P. Antiphospholipid antibodies, proteins C and S, and coagulation changes in sickle cell disease. J Lab Clin Med 1999; 134: 352–362
   PubMed Web of Science ®Google Scholar
• Francis R B. Protein S deficiency in sickle cell anemia. J Lab Clin Med 1988; 111: 571–576
   PubMed Web of Science ®Google Scholar
• Karayalcin G, Lanzkowsky P. Plasma protein C levels in children with sickle cell disease. Am J Pediatr Hematol and Oncol 1989; 11: 320–323
   PubMedGoogle Scholar
• El-Hazmi M AF, Warsy A S, Bahokim H. Blood proteins C and S in sickle cell disease. Acta Haematol 1993; 90: 114–119
   PubMed Web of Science ®Google Scholar
• Hagger D, Wolff S, Owen J, Samson D. Changes in coagulation and fibrinolysis in patients with sickle cell disease compared with healthy black controls. Blood Coag Fibrin 1995; 6: 93–99
   PubMed Web of Science ®Google Scholar
• Wright J G, Malia R, Cooper P, Thomas P, Preston F, Serjeant G R. Protein C and S in homozygous sickle cell disease. Br J Haematol 1997; 98: 627–631
   PubMed Web of Science ®Google Scholar
• Marfaing-Koka A, Boyer-Neumman C, Wolf M, Leroy-Matheron C, Cynober T, Tchernia G. Decreased protein S activity in sickle cell disease. Nouvelle Revue Francaise d'Hematologie 1993; 35: 425–430
   Google Scholar
• Cacciola E, Giustolisi R, Musso R, Longo A. Antithrombin III concentrate for treatment of chronic leg ulcers in sickle cell-beta thalassemia—a pilot study. Ann Intern Med 1989; 111: 534–536
   Google Scholar
• Onyemelukwe G C, Jibril H B. Antithrombin III deficiency in Nigerian children with sickle cell disease: possible role in the cerebral syndrome. Tropical Geo Med 1992; 44: 37–41
   Google Scholar
• Porter J B, Young L, Mackie I J, Machin S J. Sickle cell disorders and chronic intravascular hemolysis are associated with low plasma heparin cofactor II. Br J Haematol 1993; 83: 459–465
   Google Scholar
• Helley D, Girot R, Claude Guillin M, Bezeaud A. Sickle cell disease: relation between procoagulant activity of red blood cells from different phenotypes and in vivo blood coagulation activation. Br J Haematol 1997; 99: 268–272
   Google Scholar
• Setty B NY, Kulkarni S, Rao A K, Stuart M J. Fetal hemoglobin in sickle cell disease: relationship to erythrocyte phosphatidylserine exposure and coagulation activation. Blood 2000; 96: 1119–1124
   PubMed Web of Science ®Google Scholar
• Greaves M. Antiphospholipid antibodies and thrombosis. Lancet 1999; 353: 1348–1353
   PubMed Web of Science ®Google Scholar
• De Ceular, Khammasliti M A, Harris E N, Serjeant G R, Hughes G RV. Antiphospholipid antibodies in homozygous sickle cell disease. Ann Rheum Dis 1992; 51: 671–672
   Google Scholar
• Kucuk O, Gilman-Sachs A, Beaman K, Lis L V, Westerman M P. Antiphospholipid antibodies in sickle cell disease. Am J Hematol 1993; 43: 380–383
   Google Scholar
• Nsiri B, Ghazouani E, Gritili N, Mazigah C, Bayoudh F, Messaoud T. Antiphospholipid antibodies lupus anticoagulant; anticardiolipin and antiphospholipid isotypes in patients with sickle cell disease. Hematol Cell Ther 1998; 40: 107–112
   PubMedGoogle Scholar
• Ginsberg J S, Demers C, Edwards B, Bona R, Johnston M, Wong A. Acquired free protein S deficiency is associated with antiphospholipid antibodies and increased thrombin generation in patients with systemic lupus erythromatosus. Am J Med 1995; 98: 379–383
   PubMedGoogle Scholar
• Vianna J L, Khamashta M A, Ordi-Ros J, Font J, Cervera R, Lopez-Soto A. Comparison of the primary and secondary antiphospholipid syndrome: a European multicenter study of 114 patients. Am J Med 1994; 96: 3–8
   PubMed Web of Science ®Google Scholar
• Ames P PR, Tommasino C, Iannaccone L, Brillante M, Cimino R, Brancaccio V. Coagulation activation and fibrinolytic imbalance in subjects with idiopathic antiphospholipid antibodies—a crucial role for acquired free protein S deficiency. Thrombos Haemostas 1996; 76: 190–194
   PubMedGoogle Scholar
• Green D. Thrombosis in sickle cell disease. J Lab Clin Med 1999; 134: 329–330
   Google Scholar
• Merrill J T, Zhang H W, Shen C, Butman B I, Jeffries E P, Lahita R G. Enhancement of protein S anticoagulant function by B2-glycoprotein I, a major target antigen of antiphospholipid antibodies. Thrombos Haemostas 1999; 81: 748–757
   PubMedGoogle Scholar
• Wright J G, Cooper P, Malia R G, Kulozik A E, Vetter B, Thomas P, Preston F E, Serjeant G R. Activated protein C resistance in homozygous sickle cell disease. Br J Haematol 1997; 96: 854–856
   PubMed Web of Science ®Google Scholar
• Esmon N L, Smirnov M D, Esmon C T. Thrombogenic mechanisms of antiphospholipid antibodies. Thrombos Haemostas 1997; 78: 79–82
   Google Scholar
• Key N S, Shingaard A, Dandelet L, Nelson S C, Moertel C, Styles L A, Kuypers F A, Bach R R. Whole blood tissue factor procoagulant activity is elevated in patients with sickle cell disease. Blood 1998; 91: 4216–4223
   PubMed Web of Science ®Google Scholar
• Solovey A, Gui L, Key N S, Hebbel R P. Tissue factor expression by endothelial cells in sickle cell anemia. J Clin Invest 1998; 101: 1899–1904
   PubMed Web of Science ®Google Scholar
• Yeghen T, Benjamin S, Boyd G, Pumphrey C, Bevan D H. Sickle cell anemia, right atrial thrombosis and the antiphospholipid antibody. Am J Hematol 1995; 50: 46–48
   Google Scholar
• Mandori A B, Barabino G A, Lubin B H, Kuypers F A. Adherence of phosphatidylserine-exposing erythrocytes to endothelial matrix thrombospondin. Blood 2000; 95: 1293–1300
   Google Scholar
• Setty B NY, Kulkarni S, Stuart M J. Phosphatidylserine is a major determinant of sickle red blood cell-endothelial cell adhesion: in vivo and in vitro studies. 24th Annual Meeting of the National Sickle Cell Disease Program, Philadelphia, 2000; 116a
   Google Scholar
• Nemerson Y. The tissue factor pathway of blood coagulation. Semi Hematol 1992; 29: 170–176
   PubMed Web of Science ®Google Scholar
• Conkling P R, Greenberg C S, Weinberg J B. Tumor necrosis factor induces tissue factor-like activity in human leukemia cell line U937 and peripheral monocytes. Blood 1988; 72: 128
   PubMed Web of Science ®Google Scholar
• Francis R B, Haywood L J. Elevated immunoreactive tumor necrosis factor and interleukin-1 in sickle cell disease. J Natl Med Assoc 1992; 84: 611–615
   PubMed Web of Science ®Google Scholar
• Singhal A, Doherty J F, Raynes J G, McAdam K PWJ, Thomas P W, Serjeant B E, Serjeant G R. Is there an acute phase response in steady state sickle cell disease?. Lancet 1993; 341: 651
   PubMed Web of Science ®Google Scholar
• Cermak J, Key N S, Bach R R, Balla J, Jacob H S, Vercellotti G M. C-reactive protein induces human peripheral blood monocytes to synthesize tissue factor. Blood 1993; 82: 513
   PubMed Web of Science ®Google Scholar
• Solovey A, Yi Lin B S, Browne P, Choong S, Wayner E, Hebbel R P. Circulating activated endothelial cells in sickle cell disease. New Engl J Med 1997; 337: 1584–1590
   PubMed Web of Science ®Google Scholar
• Haut M J, Cowan D H, Harris J W. Platelet function and survival in sickle cell disease. J Lab Clin Med 1973; 82: 44–53
   PubMedGoogle Scholar
• Stuart M J, Stockman J A, Oski F A. Abnormalities of platelet aggregation in the vasoocclusive crisis of sickle cell anemia. J Pediatrics 1974; 85: 629–632
   Google Scholar
• Freedman M L, Karpatkin S. Elevated platelet count and megathrombocyte number in sickle cell anemia. Blood 1975; 46: 579
   PubMed Web of Science ®Google Scholar
• Gruppo R A, Glueck H I, Granger S M, Miller N A. Platelet function in sickle cell anemia. Thromb Res 1977; 10: 325–335
   Google Scholar
• Mehta P, Mehta J. Circulating platelet aggregates in sickle cell disease patients with and without vasoocclusion. Stroke 1979; 10: 464–466
   PubMedGoogle Scholar
• Kenny M W, George A J, Stuart J. Platelet hyperactivity in sickle cell disease: a consequence of hyposplenism. J Clin Pathol 1980; 33: 622
   PubMed Web of Science ®Google Scholar
• Mehta P, Mehta J. Abnormalities of platelet aggregation in sickle cell disease. J Pediatrics 1980; 96: 209–213
   PubMed Web of Science ®Google Scholar
• Mehta P. Significance of plasma beta-thromboglobulin values in patients with sickle cell disease. J Pediatrics 1980; 97: 941–944
   PubMedGoogle Scholar
• Beurling-Harbury C, Schade S. Platelet activation during pain crisis in sickle cell anemia patients. Am J Hematol 1989; 31: 237–241
   PubMed Web of Science ®Google Scholar
• Adamides S, Konstantopoulos K, Toumbis M, Douratsos D, Travlou A, Kasfiki A. A study of β-thromboglobulin and platelet factor 4 in steady state sickle cell patients. Blut 1990; 61: 245–247
   Google Scholar
• Papadimitriou C A, Travlou A, Kalos A, Douratsos D, Lali P. Study of platelet function in patients with sickle cell anemia during steady state and vasoocclusive crisis. Acta Haematol 1993; 89: 180–183
   PubMedGoogle Scholar
• Browne P V, Mosher D F, Steinberg M H, Hebbel R P. Disturbance of plasma and platelet thrombospondin levels in sickle cell disease. Am J Hematol 1996; 51: 296–301
   PubMed Web of Science ®Google Scholar
• Triadou P, Fonty F, Ambrosio A S, Cottat M C, Grot R, Cornu P. Platelet function in sickle cell disease during steady state. Nouvelle Revue Francaise d'Hematologie 1990; 32: 137–142
   PubMedGoogle Scholar
• Tomer A, Eckman J R, Vroon D H. Platelet activation and procoagulant activity in sickle cell disease. Blood 1992; 80: 11a, (abstract)
   Google Scholar
• Wun T, Paglieroni T, Tablin F, Welborn J, Nelson K, Cheung A. Platelet activation and platelet-erythrocyte aggregates in patients with sickle cell disease. J Lab Clin Med 1997; 129: 507–516
   PubMed Web of Science ®Google Scholar
• Longenecker G L, Beyers B J, Mankad V N. Platelet regulatory prostanoids and release products in sickle cell disease. Am J Hematol 1992; 40: 12–19
   PubMed Web of Science ®Google Scholar
• Setty B NY, Chen D, Stuart M J. Sickle cell vasoocclusive crisis is associated with abnormalities in the ratio of vasoconstrictor to vasodilator prostanoids. Ped Res 1995; 38: 95–102
   Google Scholar
• Foulon I, Bachir D, Galacteros F, Maclouf J. Increased in vivo production of thromboxane in patients with sickle cell disease is accompanied by an impairment of platelet functions to the thromboxane A2 agonist U46619. Arteriosclerosis and Thrombos 1993; 13: 421–426
   PubMedGoogle Scholar
• Kurantsin-Mills Ibe BO, Natta C L, Raj J U, Siegel R S, Lessin L S. Elevated urinary levels of thromboxane and prostacyclin metabolites in sickle cell disease reflects activated platelets in the circulation. Br J Hematol 1994; 87: 580–585
   Google Scholar
• Alkjaersig N, Fletcher A, Joist H, Chaplin H. Hemostatic alterations accompanying sickle cell pain crises. J Lab Clin Med 1976; 88: 440–449
   PubMed Web of Science ®Google Scholar
• Semple M J, Al Hasani S F, Kioy P, Savidge G F. A double-blind trial of ticlopidine in sickle cell disease. Thrombos Hemostas 1984; 51: 303–306
   PubMed Web of Science ®Google Scholar
• Miller R L, Verma P S, Adams R G. Studies of the kallikrein-kinin system in patients with sickle cell disease. J Nat Med. Assoc 1983; 75: 551–556
   Google Scholar
• Verma P S, Adams R G, Miller R L. Reduced plasma kininogen concentration during sickle cell crisis. Res Comm. Chem Path Pharm 1983; 41: 313–322
   Google Scholar
• Gordon E M, Klein B L, Berman B W, Strandjord S E, Simon J E, Coccia P F. Reduction of contact factors in sickle cell disease. J Pediatrics 1985; 106: 427–430
   Google Scholar
• Phillips G, Mitchell L B, Pizzo S V. Defective release of tissue plasminogen activator in patients with sickle cell anemia. Am J Hematol 1988; 29: 52–53
   Google Scholar
• Gordon P A, Breeze G R, Mann J R, Stuart J. Coagulation and fibrinolysis in sickle cell disease. J Clin Pathol 1974; 27: 485–489
   PubMedGoogle Scholar
• Francis R B. Tissue type plasminogen activator antigen and activity in sickle cell disease. J Clin Pathol 1988; 41: 490–493
   PubMedGoogle Scholar
• Phillips G, Hartman J, Keller A, Santiago M A, Pizzo S. Regulation of tissue plasminogen activator in sickle cell anemia. Am J Hematol 1990; 35: 167–170
   PubMed Web of Science ®Google Scholar
• Nsiri B, Gritli N, Bayoudh F, Messaoud T, Fattoum S, Machghoul S. Abnormalities of coagulation and fibrinolysis in homozygous sickle cell disease. Hematol Cell Ther 1996; 38: 279–284
   PubMedGoogle Scholar
• Kahn M J, Scher C, Rozans M, Michael R K, Leissinger C, Krause J. Factor V Leiden is not responsible for stroke in patients with sickling disorders and is uncommon in African-Americans with sickle cell disease. Am J Hematol 1997; 54: 12–15
   PubMed Web of Science ®Google Scholar
• Zimmerman S A, Howard T A, Whorton M R, Rosse W F, Ware R E. Thrombophilic DNA mutations as independent risk factors for stroke and avascular necrosis in sickle cell anemia. 23rd Annual Meeting of the National Sickle Cell Disease Program, San Francisco, 1999; 180
   Google Scholar
• Roszell N J, Joiner C H, Franco R S, Danton M J, Overton A, Witte D, Dougherty C, Degen J L. Effect of coagulation proteins or survival times and vasoocclusive pathology in sickle cell (SAD) Mice. 23rd Annual Meeting of the National Sickle Cell Disease Program, San Francisco, 1999; 242
   Google Scholar
• Wright J G, Malia R, Cooper P, Thomas P, Preston F E, Serjeant G R. Protein C and protein S in homozygous sickle cell disease: does hepatic dysfunction contribute to low levels?. Br J Hematol 1997; 98: 627–631
   PubMed Web of Science ®Google Scholar
• Fabry M E, Fine E, Rajanayagam Y. Demonstration of endothelial adhesion of sickle cells in vivo: a distinct role for deformable sickle cell discocytes. Blood 1992; 79: 1602
   PubMed Web of Science ®Google Scholar
• Kaul D K, Fabry M E, Nagel R L. Microvascular sites and characteristics of sickle cell adhesion to vascular endothelium in shear flow conditions: pathophysiologic implications. Proc Natl Acad Sci USA 1989; 86: 3356
   PubMed Web of Science ®Google Scholar
• Kaul D K, Fabry M E, Constantini F, Rubin E M, Nagel R L. In vivo demonstration of red cell-endothelial interactions, sickling and altered microvascular response to oxygen in the sickle transgenic mouse. J Clin Invest 1995; 96: 2845
   PubMed Web of Science ®Google Scholar
• Harlan J M. Anti-adhesion therapy in sickle cell disease. Blood 2000; 95: 365–367
   PubMed Web of Science ®Google Scholar
• Mann J R, Breeze G R, Deeble T J, Stuart J. Ancrod in sickle cell crisis. Lancet 1972; 1: 934–937
   Google Scholar
• Haddock D RW, Konotey-Ahulu F ID, Janosi M, Ankra-Badu G. Thrombosis in sickle cell pain crises? Controlled trial of ancrod (arvin) in young adults. Trop Med Hygiene 1973; 76: 274–278
   Google Scholar
• Greenberg J, Ohene-Frempong K, Halus J, Way C, Schwartz E. Trial of low dose aspirin as prophylaxis in sickle cell disease. J Pediatrics 1983; 102: 781–784
   PubMed Web of Science ®Google Scholar
• Wolters H J, ten Cate H, Thomas L L, Brandjes D P, van der Endo A, van der Heiden Y, Statius van Eps L W. Low intensity oral anticoagulation in sickle cell disease. Br J Haematol 1995; 90: 715–717
   Google Scholar
• Salvaggio J E, Arnold C A, Banov C H. Long-term anticoagulation in sickle cell disease. New Engl J Med 1963; 269: 182–186
   PubMed Web of Science ®Google Scholar
• Bridgers W H. Cerebral vascular disease accompanying sickle cell anemia. Am J Pathol 1939; 15: 353–361
   Google Scholar
• Wilimas J, Geoff J, Anderson H R, Langston J W, Thompson E. Efficacy of transfusion therapy for one to two years in patients with sickle cell disease and cerebrovascular accidents. J Pediatrics 1980; 96: 205
   PubMed Web of Science ®Google Scholar
• Samaik S, Soorya D, Kim J, Ravindranath Y, Lusher J. Periodic transfusions for sickle cell anemia and CNS infarction. Am J Dis Child 1979; 133: 1254
   Google Scholar
• Russell M O, Goldberg H I, Hudson A. Effect of transfusion therapy on arteriographic abnormalities and on recurrence of stroke in sickle cell disease. Blood 1984; 63: 162
   PubMed Web of Science ®Google Scholar
• Francis R B. Large vessel occlusion in sickle cell disease: pathogenesis, clinical consequences and therapeutic implications. Med Hypotheses 1991; 35: 88–95
   PubMed Web of Science ®Google Scholar
• Adams R J, McKie V, Hsu L, Files B, Vichinsky E, et al. Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial doppler ultrasonography. New Engl J Med 1998; 339: 5–11
   PubMed Web of Science ®Google Scholar
• Styles L, de Jong K, Vichinsky E, Lubin B, Adams R, Kuypers F. Increased RBC phosphatidylserine exposure in sickle cell disease patients at risk for stroke by transcranial doppler screening. Blood 1997; 90: 604a
   Google Scholar
• Ben Yashar V B, Barenholz Y, Hy-Am E, Rachmilewitz E A, Eldor A. Phosphatidylserine in the outer leaflet of red blood cells from β-thalassemia patients may explain the chronic hypercoagulable state and thrombotic episodes. Am J Hematol 1993; 44: 63–65
   Google Scholar
• Kuypers F A, Yuan J, Lewis R A, Snyder M, Kiefer C R, Bunyaratvej A, Fucharoen S, Ma L, Styles L, de Jong K, Schrier S L. Membrane phospholipid asymmetry in human thalassemia. Blood 1998; 91: 3044–3051
   PubMed Web of Science ®Google Scholar
• Helley D, Eldor A, Girot R, Ducrocq R, Guillin M C, Bezeaud A. Comparison of the procoagulant activity of red blood cells from patients with homozygous sickle cell disease and β-thalassemia. Thrombos Haemostas 1996; 76: 322–327
   PubMed Web of Science ®Google Scholar
• Eldor A, Durst R, Hy-Am E, Goldfarb A, Gillis S, Rachmilewitz E A, Abramov A, Maclouf J, Godefray Y C, Raucourt E, Guillin M C. A chronic hypercoagulable state in patients with β-thalassemia major is already present in childhood. BrJ Haematol 1999; 107: 739–746
   PubMed Web of Science ®Google Scholar
• Eldor A. Abnormal platelet function in β-thalassemia. Scand J Haematol 1978; 20: 447–452
   Google Scholar
• Eldor A, Krausz Y, Allan H, Snyder D, Goldfarb A, Hy-Am E, Rachmilewitz E A, Kotze H F, Heyns A P. Platelet survival in patients with β-thalassemia. Am J Hematol 1989; 32: 94–99
   PubMed Web of Science ®Google Scholar
• Eldor A, Lellouche F, Goldfarb A, Rachmilewitz E A, Maclouf J. In vivo platelet activation in β-thalassemia major reflected by increased platelet thrombox-ane urinary metabolites. Blood 1991; 77: 1749–1753
   PubMed Web of Science ®Google Scholar
• Del Principe D, Menidhelli A, Di Giulio S, De Matteis W, Cianciulli P, Papa G. PADGEM/GMP140 expression on platelet membrane from homozygous β-thalassemia patients. Br J Haematol 1993; 84: 111–117
   PubMed Web of Science ®Google Scholar
• Visudhiphan S, Ketsa-Ard K, Tumilang S, Piankijagum A. Significance of blood coagulation and platelet profiles in relation to pulmonary thrombosis in β-thalassemia/HbE. Southeast Asian J Trop Med Pub Health 1994; 25: 449–456
   PubMedGoogle Scholar
• Borgna Pignatti C, Carnellin V, Caruso V, Dore F, De Mattia D, DiPalma A, DiGeorge F, et al. Thromboembolic events in β-thalassemia major: an Italian multi-center study. Acta Haematologica 1998; 99: 76–79
   PubMed Web of Science ®Google Scholar
• Moraletti S, DeSanctis V, Gemmati D, Serino M, Mari R, Gamberini M R, Scapoli G. Thrombotic risk in thalassemic patients. J Pediatr Endocrin Metabol 1998; 11: 915–921
   Google Scholar
• Rosenberg R D, Aird W C. Vascular-bed specific hemostasis and hypercoagulable states. N EnglJ Med 1999; 340: 1555–1564
   PubMed Web of Science ®Google Scholar
• Cohen A. Sickle cell disease–new treatments, new questions. N EnglJ Med 1998; 339: 42–44
   Google Scholar