Prothrombotic changes in children with sickle cell disease: Relationships to cerebrovascular disease and transfusion

British Journal of Haematology

Volumn 103, Issue 4, 1998, Pages 1037-1044

  Liesner, R ^a   Mackie, I ^b   Cookson, J ^a   McDonald, S ^b   Chitolie, A ^b   Donohoe, S ^b   Evans, J ^c   Hann, I ^a   Machin, S ^b  

^a Gt Ormond Street Hospital NHS Trust ^*  (United Kingdom)

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

^c Qu Elizabeth Hospital for Children   (United Kingdom)

Author keywords

Children; Coagulation inhibitors; Sickle celt disease; Stroke; Thrombin generation markers

Indexed keywords

ANTITHROMBIN; HEPARIN COFACTOR II; PROTEIN S; THROMBIN;

ARTICLE; BLOOD TRANSFUSION; CHILD; FEMALE; GENETIC SUSCEPTIBILITY; HUMAN; MAJOR CLINICAL STUDY; MALE; PRIORITY JOURNAL; RISK FACTOR; SICKLE CELL ANEMIA; STROKE; THROMBOGENESIS;

ACTIVATED PROTEIN C RESISTANCE; ADOLESCENT; ANEMIA, SICKLE CELL; ANTIBODIES, ANTIPHOSPHOLIPID; ANTITHROMBINS; CEREBROVASCULAR DISORDERS; CHILD; CHILD, PRESCHOOL; FEMALE; HEPARIN COFACTOR II; HUMANS; MALE; PROTEIN C; PROTEIN S; PROTHROMBIN;

EID: 0032423610     PISSN: 00071048     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1365-2141.1998.01121.x     Document Type: Article

References (62)

1. Adams, R.J., McKie, V.C., Carl, E.M., Nichols, F.T., Perry, R., McKie, K., Figueroa, R., Litaker, M., Weiner, S.J. & Brambilla, D. (1997) Long-term stroke risk in children with sickle cell disease screened with transcranial Doppler. Annals of Neurology, 42, 699-704.
2. Adams, R.J., McKie, V.C., Hsu, L., Files, B., Vichinsky, E., Pegelow, C., Abboud, M., Gallagher, D., Kutlar, A., Nichols, F.T., Bonds, D.R. & Brambilla, D. (1998) Prevention of first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial doppler ultrasonography. New England Journal of Medicine, 339, 5-11.
3. Adams, R., McKie, V., Nichols, F., Carl, E., Zhang, D.L. McKie, K., Figueroa, R., Litaker, M., Thompson, W. & Hess, D. (1992) The use of transcranial ultrasonography to predict stroke in sickle cell disease. New England Journal of Medicine, 326, 605-610.
4. Adams, R.J., Nichols, F.T., McKie, V., McKie, K., Milner, P. & Gammal, T.E. (1988) Cerebral infarction in sickle cell anemia: mechanism based on CT and MRI. Neurology, 38, 1012-1017.
5. Andrew, M., Vegh, P., Johnston, M., Bowker, J., Ofosu, F. & Mitchell, L. (1992) Maturation of the hemostatic system during childhood. Blood, 80, 1998-2005.
6. Ballas, S.K., Larner, J., Smith, E.D., Surrey, S., Schwartz, E. & Rappaport, E.F. (1988) Rheologic predictors of the severity of the painful sickle cell crisis. Blood, 72, 1216-1223.
7. Beauchamp, N.J., Daly, M.E., Cooper, P.C., Preston, F.E. & Peake, I.R. (1994) Rapid two-stage PCR for detecting factor V G1691A mutation. (Letter). Lancet, 344, 694-695.
8. Bertina, R.M., Koeleman, B.P., Koster, T., Rosendaal, F.R., Dirven, R.J., de Ronde, H., van der Velden, P.A. & Reitsma, P.H. (1994) Mutation in blood coagulation factor V associated with resistance to activated protein C. Nature, 369, 64-67.
9. Cacciola, E., Giustolisi, R., Musso, R. & Longo, A. (1989) Antithrombin III concentrate for treament of chronic leg ulcers in sickle cell-beta thalassaemia: a pilot study. Annals of Internal Medicine, 111, 534-536.
10. Castro, O., Brambilla, D.J., Thorington, B., Reindorf, C.A., Scott, R.B., Gillette, P., Vera, J.C. & Levy, P.S. (1994) The acute chest syndrome in sickle cell disease: incidence and risk factors. The Cooperative Study of Sickle Cell Disease. Blood, 84, 643-649.
11. Charache, S. & Page, D.L. (1967) Infarction of the bone marrow in the sickle cell disorders. Annals of Internal Medicine, 67, 1195-1200.
12. Charache, S., Terrin, M.L., Moore, R.D., Dover, G.J., Barton, F.B., Eckert, S.V., McMahon, R.P., Bonds, D.R., and the Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Disease. (1995) Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. New England Journal of Medicine, 332, 1317-1322.
13. Chitolie, A., Porter, J., Machin, S.J. & Mackie, I.J. (1996) Red blood cells from normal and haemoglobinopathy patients deplete heparin cofactor II from plasma. (Abstract). Blood Coagulation and Fibrinolysis, 7, 399.
14. Chiu, D., Lubin, B., Roelofsen, B. & van Deenen, L.L. (1981) Sickled erythrocytes accelerate clotting in vitro: an effect of abnormal membrane lipid asymmetry. Blood, 58, 398-401.
15. DeBaun, M.R., Glauser, T.A., Siegel, M., Borders, J. & Lee, B. (1995) Noninvasive central nervous system imaging in sickle cell anemia: a preliminary study comparing transcranial Doppler with magnetic resonance angiography. Journal of Pediatric Hematology and Oncology, 17, 29-35.
16. De Ceulaer, K., Khamashta, M.A., Harris, E.N., Serjeant, G.R. & Hughes, G.R. (1992) Antiphospholipid antibodies in homozygous sickle cell disease. Annals of the Rheumatic Diseases, 51, 671-672.
17. de Montalembert, M., Belloy, M., Bernaudin, F., Gouraud, F., Capdeville, R., Mardini, R., Philippe, N., Jais, J., Bardakdjian, J., Ducrocq, R., Maier-Redelsperger, M., Elion, J., Labie, D. & Girot, R. (1997) Three year follow-up of hydroxyurea treatment in severely ill children with sickle cell disease. Journal of Pediatric Hematology and Oncology, 19, 313-318.
18. Devine, D.V., Kinney, T.R., Thomas, P.F., Rosse, W.F. & Greenberg, C.S. (1986) Fragment D-dimer levels: an objective marker of vasoocclusive crisis and other complications of sickle cell disease. Blood, 68, 317-519.
19. el Hazmi, M.A., Warsy, A.S. & Bahakim, H. (1993) Blood proteins C and S in sickle cell disease. Acta Haematologica, 90, 114-119.
20. Ferster, A., Vermylen, G., Cornu, G., Buyse, M., Corazza, F., Devalck, C., Fondu, P., Toppet, M. & Sariban, E. (1996) Hydroxyurea for treatment of severe sickle cell anemia: a pediatric clinical trial. Blood, 88, 1960-1964.
21. Francis, R.B., Jr (1988) Protein S deficiency in sickle cell anemia. Journal of Laboratory and Clinical Medicine, 111, 571-576.
22. Francis, R.B., Jr (1989) 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, 19, 105-111.
23. Franck, P.F., Bevers, E.M., Lubin, B.H., Comfurius, P., Chiu, D.T., Op den Kamp, J.A., Zwaal, R.F., van Deenen, L.L. & Roelofsen, B. (1985) 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. Journal of Clinical Investigation, 75, 183-190.
24. Gordon, E.M., Klein, B.L., Berman, B.W., Strandjord, S.E., Simon, J.E. & Coccia, P.F. (1985) Reduction of contact factors in sickle cell disease. Journal of Pediatrics, 106, 427-430.
25. Green, D. & Scott, J.P. (1986) Is sickle cell crisis a thrombotic event? American Journal of Hematology, 23, 317-321.
26. Gurgey, A., Mesci, L., Renda, Y., Olcay, L., Kocak, N. & Erdem, G. (1996) Factor V Q 506 mutation in children with thrombosis. American Journal of Hematology, 53, 37-39.
27. Hagger, D., Wolff, S., Owen, J. & Samson, D. (1995) Changes in coagulation and fibrinolysis in patients with sickle cell disease compared with healthy black controls. Blood Coagulation and Fibrinolysis, 6, 93-99.
28. Harris, E.N., Gharavi, A.E., Patel, S.P. & Hughes, G.R.V. (1987) Evaluation of the anticardiolipin test: report of an international workshop held 4 April 1986. Clinical and Experimental Immunology, 68, 215-222.
29. Haupt, H.M., Moore, G.W., Bauer, T.W. & Hutchins, G.M. (1982) The lung in sickle cell disease. Chest, 81, 332-337.
30. Hebbel, R.P. (1991) Beyond hemoglobin polymerisation: the red blood cell membrane and sickle disease. Blood, 77, 214-237.
31. Helley, D., Eldor, A., Girot, R., Ducrocq, R., Guillin, M.C. & Bezeaud, A. (1996) Increased procoagulant activity of red blood cells from patients with homozygous sickle cell disease and beta-thalassaemia. Thrombosis and Haemostasis, 76, 322-327.
32. Karayalcin, G., Chung, D., Pinto, P. & Lanzkowsky, P. (1984) Plasma antithrombin III levels in children with homozygous sickle cell disease (SCD). (Abstract). Pediatric Research, 18, 242A.
33. Karayalcin, G. & Lanzkowsky, P. (1989) Plasma protein C levels in children with sickle cell disease. American Journal of Pediatric Hematology and Oncology, 11, 320-323.
34. Kaul, D.K., Fabry, M.E. & Nagel, R.L. (1989) Microvascular sites and characteristics of sickle cell adhesion to vascular endothelium in shear flow conditions: pathophysiological implications. Proceedings of the National Academy of Sciences of the United States of America, 86, 3356-3360.
35. Kucuk, O., Gilman-Sachs, A., Beaman, K., Lis, L.J. & Westerman, M.P. (1993) Antiphospholipid antibodies in sickle cell disease. American Journal of Hematology, 42, 380-383.
36. Lane, P.A., O'Connell, J.L. & Marlar, R.A. (1994) Erythrocyte membrane vesicles and irreversibly sickled cells bind protein S. American Journal of Hematology, 47, 295-300.
37. Leslie, J., Langler, D., Serjeant, G.R., Serjeant, B.E., Desai, P. & Gordon, Y.B. (1975) Coagulation changes during the steady state in homozygous sickle-cell disease in Jamaica. British Journal of Haematology, 30, 159-166.
38. Loizou, S., Mccrea, J.D., Rudge, A.C., Reynolds, R., Boyle, C.C. & Harris, E.N. (1985) Measurement of anticardiolipin antibodies by enzyme linked immunosorbent assay: standardization and quantitation of results. Clinical and Experimental Immunology, 62, 739-744.
39. McNally, T., Mackie, I.J., Machin, S.J. & Isenberg, D.A. (1995) Increased levels of beta 2 glycoprotein-1 antigen and beta 2 glycoprotein-I binding antibodies are associated with a history of thromboembolic complications in patients with SLE and primary antiphospholipid syndrome. British Journal of Rheumatology, 34, 1031-1036.
40. Nowak Gottl, U., Koch, H.G., Aschka, I., Kohlhase, D., Vielhaber, H., Kurlemann, G., Oleszcuk Raschke, K., Kehl, H.G., Jurgens, H. & Schneppenheim, R. (1996) Resistance to activated protein C (APCR) in children with venous or arterial thromboembolism. British Journal of Haematology, 92, 992-998.
41. Nuss, R., Hays, T. & Manco Johnson, M. (1995) Childhood thrombosis. Pediatrics, 96, 291-294.
42. Ohene Frempong, K. (1991) Stroke in sickle cell disease: demographic, clinical, and therapeutic considerations. Seminars in Hematology, 28, 213-219.
43. Onyemelukwe, G.C. & Jibril, H.B. (1992) Antithrombin III deficiency in Nigerian children with sickle cell disease: possible role in the cerebral syndrome. Tropical Geographical Medicine, 44, 37-41.
44. Peters, M., Plaat, B.E., ten Cate, H., Wolters, H.J., Weening, R.S. & Brandjes, D.P. (1994) Enhanced thrombin generation in children with sickle cell disease. Thrombosis and Haemostasis, 71, 169-172.
45. Platt, O.S., Brambilla, D.J., Rosse, W.F., Milner, P.E., Castro, O., Steinberg, M.H. & Klug, P.P. (1994) Mortality in sickle cell disease: life expectancy and risk factors for early death. New England Journal of Medicine, 330, 1639-1644.
46. Porter, J.B., Young, L., Mackie, I.J., Marshall, L. & Machin, S.J. (1993) Sickle cell disorders and chronic intravascular haemolysis are associated with low plasma heparin cofactor II. British Journal of Haematology, 83, 459-465.
47. Powars, D., Wilson, B., Imbus, C., Pegelow, C. & Allen, J. (1978) The natural history of stroke in sickle cell disease. American Journal of Medicine, 65, 461-471.
48. Rees, D.C., Cox, M. & Clegg, J.B. (1995) World distribution of factor V Leiden. Lancet, 346, 1133-1134.
49. Rothman, S.M., Fulling, K.H. & Nelson, J.S. (1986) Sickle cell anemia and central nervous system infarction: a neuropathological study. Annals of Neurology, 20, 684-690.
50. Russell, M.O., Goldberg, H.I., Hodson, A., Kim, H.C., Halus, J., Reivich, M. & Schwartz, E. (1984) Effect of transfusion therapy on arteriographic abnormalities and on recurrence of stroke in sickle cell disease. Blood, 63, 162-169.
51. Sarnaik, S.A. & Lusher, J.M. (1982) Neurological complications of sickle cell anemia. American Journal of Pediatric Hematology and Oncology, 4, 386-394.
52. Stockman, J.A., Nigro, M.A., Mishkin, M.M. & Oski, F.A. (1972) Occlusion of large cerebral vessels in sickle-cell anemia. New England Journal of Medicine, 287, 846-849.
53. Thomas, A.N., Pattison, C. & Serjeant, G.R. (1982) Causes of death in sickle-cell disease in Jamaica. British Medical Journal: Clinical Research Edition, 285, 633-635.
54. Triplett, D.A., Stocker, K.F., Unger, G.A. & Barna, L.K. (1993) The Textarin/Ecarin ratio: a confirmatory test for lupus anticoagulants. Thrombosis and Haemostasis, 70, 925-951.
55. Vichinsky, E., Styles, L.A., Colangelo, L.H., Wright, E.C., Castro, O. & Nickerson, B. (1997) Acute chest syndrome in sickle cell disease. Blood, 89, 1787-1792.
56. Wiznitzer, M., Ruggieri, P.M., Masaryk, T.J., Ross, J.S., Modic, M.T. & Berman, B. (1990) Diagnosis of cerebrovascular disease in sickle cell anemia by magnetic resonance angiography. Journal of Pediatrics. 117, 551-555.
57. Wolters, H.J., ten Cate, H., Thomas, L.L., Brandjes, D.P., van der Ende, A., van der Heiden, Y. & Statius van Eps, L.W. (1995) Low-intensity oral anticoagulation in sickle-cell disease reverses the prethrombotic state: promises for treatment? British Journal of Haematology, 90, 715-717.
58. Wood, B.L., Gibson, D.F. & Tait, J.F. (1996) Increased erythrocyte phosphatidylserine exposure in sickle cell disease: flow-cytometric measurement and clinical associations. Blood. 88, 1873-1880.
59. Woodhams, B.J. (1988) The simultaneous measurement of total and free protein S by ELISA. Thrombosis Research, 50, 213-220.
60. Wright, J.G., Cooper, P., Malia, R.G., Kulozik, A.E., Vetter, B., Thomas, P., Preston, F.E. & Serjeant, G.R. (1997a) Activated protein C resistance in homozygous sickle cell disease. British Journal of Haematology, 96, 854-856.
61. Wright, J.G., Malia, R.G., Cooper, P., Thomas, P., Preston, F.E. & Serjeant, G.R. (1997b) Protein C and protein S in homozygous sickle cell disease: does hepatic dysfunction contribute to low levels? British Journal of Haematology, 98, 627-631.
62. Yeghen, T., Benjamin, S., Boyd, O., Pumphrey, C. & Bevan, D.H. (1995) Sickle cell anemia, right atrial thrombosis, and the antiphospholipid antibody. American Journal of Hematology, 50, 46-48.