Thrombosis in Paroxysmal Nocturnal Hemoglobinuria at a glance: A clinical review

Current Vascular Pharmacology

Volumn 6, Issue 4, 2008, Pages 347-353

  Ziakas, Panayiotis D ^a   Poulou, Loukia S ^b   Pomoni, Anastasia ^c  

^a HYGEIA HOSPITAL   (Greece)

^b General Oncology Hospital Agioi Anargyroi   (Greece)

^c LAUSANNE UNIVERSITY HOSPITAL   (Switzerland)

Author keywords

Diagnosis; Eculizumab; Management; Paroxysmal nocturnal hemoglobinuria; Thrombosis

Indexed keywords

ECULIZUMAB; FIBRINOLYTIC AGENT; LOW MOLECULAR WEIGHT HEPARIN; PROCOAGULANT; WARFARIN; ANTICOAGULANT AGENT;

ANGIOPLASTY; ANTICOAGULATION; ARTERY THROMBOSIS; BONE MARROW DEPRESSION; BONE MARROW TRANSPLANTATION; CELL DAMAGE; CEREBRAL VEIN THROMBOSIS; CEREBROVASCULAR DISEASE; COMPUTER ASSISTED TOMOGRAPHY; DIAGNOSTIC IMAGING; ENDOTHELIUM; FIBRINOLYSIS; FOLLOW UP; HEMOLYSIS; HEMOLYTIC ANEMIA; HEPATIC VEIN; HUMAN; HYPERCOAGULABILITY; LUNG EMBOLISM; MESENTERIC VEIN; MORBIDITY; MORTALITY; NUCLEAR MAGNETIC RESONANCE IMAGING; PAROXYSMAL NOCTURNAL HEMOGLOBINURIA; PATHOGENESIS; PREGNANCY; PRIMARY PREVENTION; REVIEW; SECONDARY PREVENTION; SHUNTING; THROMBOCYTE; THROMBOEMBOLISM; THROMBOPHILIA; THROMBOSIS; VEIN THROMBOSIS; VISCERAL THROMBOSIS; FEMALE; FIBRINOLYTIC THERAPY; PATHOLOGY; PERIPHERAL OCCLUSIVE ARTERY DISEASE; PREGNANCY COMPLICATION; TREATMENT OUTCOME; VASCULAR SURGERY;

ANTICOAGULANTS; ARTERIAL OCCLUSIVE DISEASES; BONE MARROW TRANSPLANTATION; FEMALE; HEMOGLOBINURIA, PAROXYSMAL; HUMANS; PREGNANCY; PREGNANCY COMPLICATIONS, CARDIOVASCULAR; PULMONARY EMBOLISM; THROMBOLYTIC THERAPY; THROMBOSIS; TREATMENT OUTCOME; VASCULAR SURGICAL PROCEDURES; VENOUS THROMBOSIS;

EID: 55449085526     PISSN: 15701611     EISSN: None     Source Type: Journal    
DOI: 10.2174/157016108785909742     Document Type: Review

References (66)

1. Strubing P. Paroxysmalhaemoglobinurie. Dtsch Med Wochenschr 1882; 8: 1-17.
2. Ham TH. Chronic hemolytic anemia with paroxysmal nocturnal hemoglobinuria. A study of the mechanism of hemolysis in relation to acid-base equilibrium. N Engl J Med 1937; 217: 215-7.
3. Hartmann RC, Jenkins DE. The 'sugar-water test' for paroxysmal nocturnal hemoglobinuria. N Engl J Med 1965; 275: 155-7.
4. Hall SE, Rosse WF. The use of monoclonal antibodies and flow cytometry in the diagnosis of paroxysmal nocturnal hemoglobinuria. Blood 1996; 87: 5332-40.
5. Dameshek W. Riddle: what do aplastic anemia, paroxysmal nocturnal hemoglobinuria [PNH] and "hypoplastic" leukemia have in common? Blood 1967; 30: 251-4.
6. Crosby WH. PNH: Relation of the clinical manifestations to underlying pathogenic mechanisms. Blood 1953; 8: 769-812.
7. Ploug M, Plesner T, Ronne E, Ellis V, Høyer-Hansen G, Hansen NE, et al. The receptor for urokinase-type plasminogen activator is deficient on peripheral blood leukocytes in patients with paroxysmal nocturnal hemoglobinuria. Blood 1992; 79: 1447-55.
8. Hugel B, Socie G, Vu T, Toti F, Gluckman E, Freyssinet JM, et al. Elevated levels of circulating procoagulant microparticles in patients with paroxysmal nocturnal hemoglobinuria and aplastic anemia. Blood 1999; 93: 3451-6.
9. Liebman HA, Feinstein DI. Thrombosis in patients with paroxysmal nocturnal hemoglobinuria is associated with markedly elevated plasma levels of leukocyte-derived tissue factor. Thromb Res 2003; 111: 235-8.
10. Asch AS, Kinoshita T, Jaffe EA, Nussenzweig V. Decay accelerating factor is present on cultured human umbilical vein endothelial cells. J Exp Med 1986; 163: 221-6.
11. Rother RP, Bell L, Hillmen P, Gladwin MT. The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease. JAMA 2005; 293: 1653-62.
12. Schäfer A, Wiesmann F, Neubauer S, Eigenthaler M, Bauersachs J, Channon KM. Rapid regulation of platelet activation in vivo by nitric oxide. Circulation 2004; 109:1819-22.
13. Reiter CD, Wang X, Tanus-Santos JE, Hogg N, Cannon RO 3rd, Schechter AN, et al. Cell-free hemoglobin limits nitric oxide bioavailability in sickle-cell disease. Nat Med 2002; 8: 1383-9.
14. Olson JS, Foley EW, Rogge C, Tsai AL, Doyle MP, Lemon DD. NO scavenging and the hypertensive effect of hemoglobin-based blood substitutes. Free Radic Biol Med 2004; 36: 685-97. http://bloodjournal.hematologylibrary.org/cgi/external_ref?access_num= 10.1038/nm799&link_type=DOI
15. Hall C, Richards S, Hillmen P. Primary prophylaxis with warfarin prevents thrombosis in paroxysmal nocturnal hemoglobinuria [PNH]. Blood 2003; 102: 3587-91.
16. Moyo VM, Mukhina GL, Garrett ES, Brodsky RA. Natural history of paroxysmal nocturnal haemoglobinuria using modern diagnostic assays. Br J Haematol 2004; 126: 133-8.
17. Ziakas PD, Poulou LS, Rokas GI, Bartzoudis D, Voulgarelis M. Thrombosis in Paroxysmal Nocturnal Hemoglobinuria: sites, risks, outcome. An overview. J Thromb Haemost 2007; 5: 642-5.
18. Hirshberg B, Shouval D, Fibach E, Friedman G, Ben-Yehuda D. Flow cytometric analysis of autonomous growth of erythroid precursors in liquid culture detects occult polycythemia vera in the Budd-Chiari syndrome. J Hepatol 2000; 32: 574-8.
19. Mahmoud AE, Elias E, Beauchamp N, Wilde JT. Prevalence of the factor V Leiden mutation in hepatic and portal vein thrombosis. Gut 1997; 40: 798-800.
20. Das M, Carroll SF. Antithrombin III deficiency: an etiology of Budd-Chiari syndrome. Surgery 1985; 97: 242-6.
21. Bourliere M, Le Trent YP, Amoux D, Castellani P, Bordigoni L, Maillot A, et al. Acute Budd-Chiari syndrome with hepatic failure and obstruction of the inferior vena cava as presenting manifestations of hereditary protein C deficiency. Gut. 1990; 31: 949-52.
22. Ganguli SC, Ramzan NN, McKusick MA, Andrews JC, Phyliky RL, Karnath PS. Budd-Chiari syndrome in patients with hematological disease: a therapeutic challenge. Hepatology 1998; 27: 1157-61.
23. Hauser AC, Brichts, A, Pabinger-Fasching I, Jager U. Fibrinolytic therapy with rt-PA in a patient with paroxysmal nocturnal hemoglobinuria and Budd-Chiari syndrome. Ann Hematol 2003; 82: 299-302.
24. McMullin MF, Hillmen P, Jackson J, Ganly P, Luzzatto L. Tissue plasminogen activator for hepatic vein thrombosis in paroxysmal nocturnal haemoglobinuria. J Intern Med 1994; 235: 85-9.
25. McKee CM, Mayne EE, Crothers JG, Callender ME. Budd-Chiari syndrome treated with acylated streptokinase-plasminogen complex. J R Soc Med 1985; 78: 768-9.
26. Raju GS, Felver M, Olin JW, Satti SD. Thrombolysis for acute Budd-Chiari syndrome: case report and literature review. Am J Gastmenterol 1996; 91: 1262-3.
27. Wang ZG, Zhang FJ, Yi MQ, Qiang LX Evolution of management for Budd-Chiari syndrome: a team's view from 2564 patients. ANZ J Surg 2005; 75: 55-63.
28. Senzolo M, Cholongitas EC, Patch D, Burroughs AK. Update on the classification, assessment of prognosis and therapy of Budd-Chiari syndrome. Nat Chn Pract Gastroenterol Hepatol 2005; 2: 182-90.
29. Plessier A, Sibert A, Consigny Y, Hakime A, Zappa M, Denninger MH, et al. Aiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome. Hepatology 2006; 44: 1308-16.
30. Kumar KS, Malet PF, Rutherford C. Paroxysmal nocturnal hemoglobinuria with no evidence of hemolysis presenting as Budd-Chiari syndrome. Dig Dis Sci 2000; 45: 2238-9.
31. Bahr MJ, Schubert J, Bleck JS, Tietge UJ, Boozari B, Schmidt RE, et al. Recurrence of Budd-Chiari syndrome after liver transplantation in paroxysmal nocturnal hemoglobinuria. Transpl Int 2003; 16: 890-4.
32. Kugelmas M. Budd-Chiari syndrome. Treatment options and the value of liver transplantation. Hepatogastroenterology 1998; 45: 1381-6.
33. Graham ML, Rosse WF, Halperin EC, Miller CR, Ware RE. Resolution of Budd Chiari syndrome following bone marrow transplantation for paroxysmal nocturnal hoemoglobinuria. Br J Haematol 1996; 92: 707-10.
34. Gayer G, Zandman-Goddard G, Raanani P, Hertz M, Apter S. Widespread abdominal venous thrombosis in paroxysmal nocturnal hemoglobinuria diagnosed on CT. Abdom Imaging 2001; 26: 414-9.
35. Tsatalas C, Margaritis D, Pantelidou D, Kotsianidis I, Karayiannakis AJ, Spanoudakis E, et al. Splenectomy for massive splenic infarction unmasks paroxysmal nocturnal hemoglobinuria. Acta Haematol 2003; 110: 193-6.
36. Tomizuka H, Hatake K, Kitagawa S, Yamashita Y, Arai H, Miura Y. Portal vein thrombosis in paroxysmal nocturnal haemoglobinuria. Acta Haematol 1999; 101: 149-52.
37. Brunaud L, Antunes L, Collinet-Adler S, Marchal F, Ayav A, Bresler L, et al. Acute mesenteric venous thrombosis: case for nonoperative management. J Vasc Surg 2001; 34:673-9.
38. Morasch MD, Ebaugh JL, Chiou AC, Matsumura JS, Pearce WH, Yao JS. Mesenteric venous thrombosis: a changing clinical entity. J Vasc Surg 2001; 34: 680-4.
39. Zhang J, Duan ZQ, Song QB, Luo YW, Xin SJ, Zhang Q. Acute mesenteric-yenous thrombosis: a better outcome achieved through improved imaging techniques and a changed policy of clinical management. Eur J Vasc Endovasc Surg 2004; 28: 329-34.
40. Grisham A, Lohr J, Guenther JM, Engel AM. Deciphering mesenteric venous thrombosis: imaging and treatment. Vasc Endovascular Surg 2005; 39: 473-9.
41. Mathieu D, Rahmouni A, Villeneuve P, Anglade MC, Rochant H, Vasile N. Impact of magnetic resonance imaging on the diagnosis of abdominal complications of paroxysmal nocturnal hemoglobinuria. Blood 1995; 85: 3283-8.
42. Roubidoux MA. MR of the kidneys, liver, and spleen in paroxysmal nocturnal hemoglobinuria. Abdom Imaging 1994; 19:168-73.
43. Kerr AB, Pauly JM, Hu BS, Li KC, Hardy CJ, Meyer CH, et al. Real-time interactive MRI on a conventional scanner. Magn Reson Med 1997; 38: 355-67.
44. Holsinger AE, Wright RC, Riederer SJ, Firzaneh F, Grimm RC, Maier JK. Real-time interactive magnetic resonance imaging. Magn Reson Med 1990; 14: 547-53.
45. Shirkhoda A, Konez O, Shetty AN, Bis KG, Ellwood RA, Kirsch MJ. Mesenteric circulation: three-dimensional MR angiography with a gadolinium-enhanced multiecho gradient-echo technique. Radiology 1997; 202: 257-261.
46. Bradbury MS, Kavanagh PV, Bechtold RE, Chen MY, Ott DJ, Regan JD, et al. Mesenteric venous thrombosis: diagnosis and non-invasive imaging. Radiographics 2002; 22: 527-41.
47. Donhowe SP, Lazaro RP. Dural sinus thrombosis in paroxysmal nocturnal hemoglobinuria. Clin Neurol Neurosurg 1984; 86: 149-52.
48. Poulou LS, Vakrinos G, Pomoni A, Michalakis K, Karianakis G, Voulgarelis M, et al. Stroke in paroxysmal nocturnal haemoglobinuria: patterns of disease and outcome. Thromb Haemost 2007; 98: 699-701.
49. Van Vleymen B, Dehaene I, Van Hoof A, Pattyn G. Cerebral venous thrombosis in paroxysmal nocturnal haemoglobinuria. Acta Neurol Belg 1987; 87: 80-7.
50. Clark DA, Butler SA, Braren V, Hartmann RC, Jenkins DE Jr. The kidneys in paroxysmal nocturnal hemoglobinuria Blood 1981; 57: 83-9.
51. Staab EV, Hartman RC, Parrott JA. Liver imaging in the diagnosis of hepatic venous thrombosis in paroxysmal nocturnal hemoglobinuria. Radiology 1975; 117: 341-8.
52. Heller PG, Grinberg AR, Lencioni M, Molina MM, Roncoroni AJ. Pulmonary hypertension in paroxysmal nocturnal hermoglobinuria. Chest 1992; 102: 642-3.
53. Audebert HJ, Planck J, Eisenburg M, Schrezenmeier H, Haberl RL. Cerebral ischemic infarction in paroxysmal nocturnal hemoglobinuria report of 2 cases and updated review of 7 previously published patients. J Neurol 2005; 252: 1379-86.
54. Hillmen P, Lewis SM, Bessler M, Luzzatto L, Dacie JV. Natural history of paroxysmal nocturnal hemoglobinuria. N Engl J Med 1995; 333: 1253-8.
55. Steinberg D, Carvalho AC, Chesney CM, Colman RW. Platelet hypersensitivity and intravascular coagulation in paroxysmal nocturnal hemoglobinuria. Am J Med 1975; 59: 845-50.
56. McBane RD 2nd, Panneton JM, Bate WW. Aortic thrombosis as a complication of paroxysmal nocturnal hemoglobinuria. Circulation 2001; 104: E1-2.
57. Yamazaki T, Suzuki K, Sumi M, Yanaka K, Kojima H, Matsumura A. Cerebral embolism as a complication of paroxysmal nocturnal hemoglobinuria. Eur Neurol 2005; 53: 217-20.
58. Bjorge L, Ernst P, Haram KO. Paroxysmal nocturnal hemoglobinuria in pregnancy. Acta Obstet Gynecol Scand 2003; 82: 1067-71.
59. Ray JG, Burrows RF, Ginsberg JS, Burrows EA. Paroxysmal nocturnal hemoglobinuria and the risk of venous thrombosis: review and recommendations for management of the pregnant and non-pregnant patient. Haemostasis 2000; 30: 103-17.
60. Tichelli A, Socie G, Marsh J Barge R, Frickhofen N, McCann S, et al.; European Group for Blood and Marrow Transplantation Severe Aplastic Anaemia Working Party. Outcome of pregnancy and disease course among women with aplastic anemia treated with immunosuppression. Ann Intern Med 2002; 137: 164-72.
61. Valla D, Dhumeaux D, Babany G Hillon P, Rueff B, Rochant H, et al. Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. A spectrum from asymptomatic occlusion of hepatic venules to fatal Budd-Chiari syndrome. Gastroenterology 1987; 93: 569-75.
62. Poulou LS, Xila V, Rokas GI, Bartzoudis D, Karianakis G, Ziakas PD. Temporal trends in mortality rates from visceral vein thrombosis in paroxysmal nocturnal hemoglobinuria: An optimistic view. Thromb Haemost 2008; 99: 642-5.
63. Sholar PW, Bell WR. Thrombolytic: therapy for inferior vena cava. thrombosis in paroxysmal nocturnal hemoglobinuria. Ann Intern Med 1985; 103: 539-41.
64. Brodsky RA, Young NS, Antonioli E, Risitano AM, Schrezenmeier H, Schubert J, et al. Multicenter phase III study of the complement inhibitor eculizumab for the treatment of patients with paroxysmal nocturnal hemoglobinuria. Blood 2008; 111: 1840-7.
65. Hillmen P, Muus P, Dührsen U, Risitano AM, Schubert J, Luzzatto L, et al. Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria. Blood 2007; 110: 4123-8.
66. Saso R, Marsh J, Cevreska L, Szer J, Gale RP, Rowlings PA, et al. Bone marrow transplants for paroxysmal nocturnal haemoglobinuria. Br J Haematol 1999; 104: 392-6.