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. 2010 Jan 14:529–569. [Article in German] doi: 10.1007/978-3-642-01544-1_35

Komplexe Gerinnungsstörungen

B Lämmle 3, J A Kremer Hovinga 3, W Rath 4, B Pötzsch 5, K Madlener 6, E Lengfelder 7, T Neuhaus 8, D U Bayraktar 9
Editors: Bernd Pötzsch1, Katharina Madlener2
PMCID: PMC7123555

Zusammenfassung

Die thrombotisch-thrombozytopenische Purpura (TTP) und das hämolytischurämische Syndrom (HUS) sind thrombotische Mikroangiopathien, gekennzeichnet durch eine Endothelzellschädigung mit nachfolgender Bildung von Thromben in der Mikrozirkulation mit intravasaler Hämolyse und Thrombozytopenie. Ischämische Organdysfunktionen im Gehirn, den Nieren und anderen Organen Prägen das klinische Bild. Während bei Erwachsenen das Auftreten einer neurologischen Symptomatik zur Diagnose TTP führt, wird bei Kindern mit dem Leitsymptom Nierenversagen die Diagnose HUS gestellt.

Literatur

  1. Allford S.L., Hunt B.J., Rose P., Machin S.J. Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br J Haematol. 2003;120(4):556–73. doi: 10.1046/j.1365-2141.2003.04049.x. [DOI] [PubMed] [Google Scholar]
  2. Amorosi E.L., Ultmann J.E. Thrombotic thrombocytopenic purpura: report of 16 cases and review of the literature. Medicine (Baltimore) 1966;45:139–59. [Google Scholar]
  3. Asada Y., Sumiyoshi A., Hayashi T., Suzumiya J., Kaketani K. Immunohistochemistry of vascular lesion in thrombotic thrombocytopenic purpura, with special reference to factor VIII related antigen. Thromb Res. 1985;38(5):469–79. doi: 10.1016/0049-3848(85)90180-X. [DOI] [PubMed] [Google Scholar]
  4. Atkinson J.P., Liszewski M.K., Richards A., Kavanagh D., Moulton E.A. Hemolytic uremic syndrome: an example of insufficient complement regulation on self-tissue. Ann N Y Acad Sci. 2005;1056:144–52. doi: 10.1196/annals.1352.032. [DOI] [PubMed] [Google Scholar]
  5. Banno F., Kokame K., Okuda T., Honda S., Miyata S., Kato H., Tomiyama Y., Miyata T. Complete deficiency in ADAMTS13 is prothrombotic, but it alone is not sufficient to cause thrombotic thrombocytopenic purpura. Blood. 2006;107(8):3161–6. doi: 10.1182/blood-2005-07-2765. [DOI] [PubMed] [Google Scholar]
  6. Barbot J., Costa E., Guerra M., et al. Ten years of prophylactic treatment with fresh-frozen plasma in a child with chronic relapsing thrombotic thrombocytopenic purpura as a result of a congenital deficiency of von Willebrand factor-cleaving protease. Br J Haematol. 2001;113(3):649–51. doi: 10.1046/j.1365-2141.2001.02808.x. [DOI] [PubMed] [Google Scholar]
  7. Bell W.R., Braine H.G., Ness P.M., Kickler T.S. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med. 1991;325(6):398–403. doi: 10.1056/NEJM199108083250605. [DOI] [PubMed] [Google Scholar]
  8. Bennett C.L., Weinberg P.D., Rozenberg-Ben-Dror K., Yarnold P.R., Kwaan H.C., Green D. Thrombotic thrombocytopenic purpura associated with ticlopidine. A review of 60 cases. Ann Intern Med. 1998;128(7):541–4. doi: 10.7326/0003-4819-128-7-199804010-00004. [DOI] [PubMed] [Google Scholar]
  9. Bernardo A., Ball C., Nolasco L., Moake J.F., Dong J.F. Effects of inflammatory cytokines on the release and cleavage of the endothelial cell-derived ultralarge von Willebrand factor multimers under flow. Blood. 2004;104(1):100–6. doi: 10.1182/blood-2004-01-0107. [DOI] [PubMed] [Google Scholar]
  10. Bianchi V., Robles R., Alberio L., Furlan M., Lämmle B. Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura. Blood. 2002;100(2):710–3. doi: 10.1182/blood-2002-02-0344. [DOI] [PubMed] [Google Scholar]
  11. Byrnes J.J., Khurana M. Treatment of thrombotic thrombocytopenic purpura with plasma. N Engl J Med. 1977;297(25):1386–9. doi: 10.1056/NEJM197712222972507. [DOI] [PubMed] [Google Scholar]
  12. Chandler W.L., Jelacic S., Boster D.R., Ciol M.A., Williams G.D., Watkins S.L., Igarashi T., Tarr P.I. Prothrombotic coagulation abnormalities preceding the hemolytic-uremic syndrome. N Engl J Med. 2002;346(1):23–32. doi: 10.1056/NEJMoa011033. [DOI] [PubMed] [Google Scholar]
  13. Dent J.A., Berkowitz S.D., Ware J., Kasper C.K., Ruggeri Z.M. Identification of a cleavage site directing the immunochemical detection of molecular abnormalities in type IIA von Willebrand factor. Proc Natl Acad Sci U S A. 1990;87(16):6306–10. doi: 10.1073/pnas.87.16.6306. [DOI] [PMC free article] [PubMed] [Google Scholar]
  14. Donadelli R., Banterla F., Galbusera M., et al. In-vitro and in-vivo consequences of mutations in the von Willebrand factor cleaving protease ADAMTS13 in thrombotic thrombocytopenic purpura. Thromb Haemost. 2006;96(4):454–64. [PubMed] [Google Scholar]
  15. Dong J.F., Moake J.L., Nolasco L., Bernardo A., Arceneaux W., Shrimpton C.N., Schade A.J., McIntire L.V., Fujikawa K., Lopez J.A. ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions. Blood. 2002;100(12):4033–9. doi: 10.1182/blood-2002-05-1401. [DOI] [PubMed] [Google Scholar]
  16. Dragon-Durey M.A., Fremeaux-Bacchi V., et al. Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases. J Am Soc Nephrol. 2004;15(3):787–95. doi: 10.1097/01.ASN.0000115702.28859.A7. [DOI] [PubMed] [Google Scholar]
  17. Dragon-Durey M.A., Loirat C., Cloarec S., Macher M.A., Blouin J., Nivet H., Weiss L., Fridman W.H., Fremeaux-Bacchi V. Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome. J Am Soc Nephrol. 2005;16(2):555–63. doi: 10.1681/ASN.2004050380. [DOI] [PubMed] [Google Scholar]
  18. Dundas S., Murphy J., Soutar R.L., Jones G.A., Hutchinson S.J., Todd W.T. Effectiveness of therapeutic plasma exchange in the 1996 Lanarkshire Escherichia coli O157: H7 outbreak. Lancet. 1999;354(9187):1327–30. doi: 10.1016/S0140-6736(99)01251-9. [DOI] [PubMed] [Google Scholar]
  19. Fakhouri F., Vernant J.P., Veyradier A., et al. Efficiency of curative and prophylactic treatment with rituximab in ADAMTS13-deficient thrombotic thrombocytopenic purpura: a study of 11 cases. Blood. 2005;106(6):1932–37. doi: 10.1182/blood-2005-03-0848. [DOI] [PubMed] [Google Scholar]
  20. Fontana S., Kremer Hovinga J.A., Studt J.D., Alberio L., Lämmle B., Mansouri Taleghani B. Plasma therapy in thrombotic thrombocytopenic purpura: review of the literature and the Bern experience in a subgroup of patients with severe acquired ADAMTS-13 deficiency. Semin Hematol. 2004;41(1):48–59. doi: 10.1053/j.seminhematol.2003.10.010. [DOI] [PubMed] [Google Scholar]
  21. Fujikawa K., Suzuki H., McMullen B., Chung D. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood. 2001;98(6):1662–6. doi: 10.1182/blood.V98.6.1662. [DOI] [PubMed] [Google Scholar]
  22. Furlan M., Lämmle B. Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: the role of von Willebrand factor-cleaving protease. Best Pract Res Clin Haematol. 2001;14(2):437–54. doi: 10.1053/beha.2001.0142. [DOI] [PubMed] [Google Scholar]
  23. Furlan M., Robles R., Galbusera M., et al. Von Willebrand factorcleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med. 1998;339(22):1578–84. doi: 10.1056/NEJM199811263392202. [DOI] [PubMed] [Google Scholar]
  24. Furlan M., Robles R., Solenthaler M., Lämmle B. Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura. Blood. 1998;91(8):2839–46. [PubMed] [Google Scholar]
  25. Furlan M., Robles R., Solenthaler M., Wassmer M., Sandoz P., Lämmle B. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood. 1997;89(9):3097–103. [PubMed] [Google Scholar]
  26. Furlan M., Robles R., Lämmle B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood. 1996;87(10):4223–34. [PubMed] [Google Scholar]
  27. Gasser C., Gautier E., Steck A., Siebenmann R.E., Oechslin R. Hämolytisch-urämische Syndrome: bilaterale Nierenrindennekrosen bei akuten erworbenen hämolytischen Anämien. Schweiz Med Wochenschr. 1955;85:905–909. [PubMed] [Google Scholar]
  28. George J.N., Kremer Hovinga J.A., Terrell D.R., Vesely S.K., Lämmle B. The Oklahoma Thrombotic Thrombocytopenic Purpura-Hemolytic Uremic Syndrome Registry: the Swiss connection. Eur J Haematol. 2008;80(4):277–86. doi: 10.1111/j.1600-0609.2008.01040.x. [DOI] [PubMed] [Google Scholar]
  29. George J.N. Clinical practice. Thrombotic thrombocytopenic purpura. N Engl J Med. 2006;354(18):1927–35. doi: 10.1056/NEJMcp053024. [DOI] [PubMed] [Google Scholar]
  30. George J.N. How I treat patients with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Blood. 2000;96(4):1223–29. [PubMed] [Google Scholar]
  31. Gerritsen H.E., Robles R., Lämmmle B., Furlan M. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood. 2001;98(6):1654–61. doi: 10.1182/blood.V98.6.1654. [DOI] [PubMed] [Google Scholar]
  32. Goicoechea de Jorge E., Harris C.L., Esparza-Gordillo J., et al. Gain-offunction mutations in complement factor B are associated with atypical hemolytic uremic syndrome. Proc Natl Acad Sci USA. 2007;104(1):240–5. doi: 10.1073/pnas.0603420103. [DOI] [PMC free article] [PubMed] [Google Scholar]
  33. Griffin P.M., Tauxe R.V. The epidemiology of infections caused by Escherichia coli O157: H7, other enterohemorrhagic E. coli, and the associated hemolytic uremic syndrome. Epidemiol Rev. 1991;13:60–98. doi: 10.1093/oxfordjournals.epirev.a036079. [DOI] [PubMed] [Google Scholar]
  34. Gunther K., Garizio D., Nesara P. ADAMTS13 activity and the presence of acquired inhibitors in human immunodeficiency virus-related thrombotic thrombocytopenic purpura. Transfusion. 2007;47(9):1710–6. doi: 10.1111/j.1537-2995.2007.01346.x. [DOI] [PubMed] [Google Scholar]
  35. Hommais A., Rayes J., Houllier A., Obert B., Legendre P., Veyradier A., Girma J.P., Ribba A.S. Molecular characterization of four ADAMTS13 mutations responsible for congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) Thromb Haemost. 2007;98(3):593–9. [PubMed] [Google Scholar]
  36. Hosler G.A., Cusumano A.M., Hutchins G.M. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are distinct pathologic entities. A review of 56 autopsy cases. Arch Pathol Lab Med. 2003;127(7):834–9. doi: 10.5858/2003-127-834-TTPAHU. [DOI] [PubMed] [Google Scholar]
  37. Hulstein J.J., de Groot P.G., Silence K., Veyradier A., Fijnheer R., Lenting P.J. A novel nanobody that detects the gain-of-function phenotype of von Willebrand factor in ADAMTS13 deficiency and von Willebrand disease type 2B. Blood. 2005;106(9):3035–42. doi: 10.1182/blood-2005-03-1153. [DOI] [PubMed] [Google Scholar]
  38. Hulstein J.J., van Runnard Heimel P.J., Franx A., Lenting P.J., Bruinse H.W., Silence K., de Groot P.G., Fijnheer R. Acute activation of the endothelium results in increased levels of active von Willebrand factor in hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome. J Thromb Haemost. 2006;4(12):2569–75. doi: 10.1111/j.1538-7836.2006.02205.x. [DOI] [PubMed] [Google Scholar]
  39. Kappers-Klunne M.C., Wijermans P., Fijnheer R., Croockewit A.J., van der Holt B., de Wolf J.T., Lowenberg B., Brand A. Splenectomy for the treatment of thrombotic thrombocytopenic purpura. Br J Haematol. 2005;130(5):768–76. doi: 10.1111/j.1365-2141.2005.05681.x. [DOI] [PubMed] [Google Scholar]
  40. Kavanagh D., Goodship T.H. Update on evaluating complement in hemolytic uremic syndrome. Curr Opin Nephrol Hypertens. 2007;16(6):565–71. doi: 10.1097/MNH.0b013e3282f0872f. [DOI] [PubMed] [Google Scholar]
  41. Kinoshita S., Yoshioka A., Park Y.D., et al. Upshaw-Schulman-Syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura. Int J Hematol. 2001;74(1):101–8. doi: 10.1007/BF02982558. [DOI] [PubMed] [Google Scholar]
  42. Kokame K., Nobe Y., Kokubo Y., Okayama A., Miyata T. FRETS-VWF73, a first fluorogenic substrate for ADAMTS13 assay. Br J Haematol. 2005;129(1):93–100. doi: 10.1111/j.1365-2141.2005.05420.x. [DOI] [PubMed] [Google Scholar]
  43. Kokame K., Matsumoto M., Soejima K., et al. Mutations and common polymorphisms in ADAMTS13 gene responsible for von Willebrand factor-cleaving protease activity. Proc Natl Acad Sci USA. 2002;99(18):11902–7. doi: 10.1073/pnas.172277399. [DOI] [PMC free article] [PubMed] [Google Scholar]
  44. Kremer Hovinga J.A., Zeerleder S., Kessler P., et al. ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock. J Thromb Haemost. 2007;5(11):2284–90. doi: 10.1111/j.1538-7836.2007.02743.x. [DOI] [PubMed] [Google Scholar]
  45. Kremer Hovinga J.A., Mottini M., Lämmle B. Measurement of ADAMTS-13 activity in plasma by the FRETS-VWF73 assay: comparison with other assay methods. J Thromb Haemost. 2006;4(5):1146–8. doi: 10.1111/j.1538-7836.2006.01904.x. [DOI] [PubMed] [Google Scholar]
  46. Kremer Hovinga JA, Zahnd R, George JN et al. (2006b) Evidence for a pathophysiological role of anti-ADAMTS13 antibodies despite the presence of normal ADAMTS13 activity and presumption of an epitope spreading over time in recurrent thrombotic thrombocytopenic purpura (TTP). In: 48th Annual Meeting of the American Society of Hematology 2006 Orlando, Florida: Blood 2006, Abstract #1067
  47. Kremer Hovinga J.A., Studt J.D., Alberio L., Lämmle B. von Willebrand factor-cleaving protease (ADAMTS-13) activity determination in the diagnosis of thrombotic microangiopathies: the Swiss experience. Semin Hematol. 2004;41(1):75–82. doi: 10.1053/j.seminhematol.2003.10.008. [DOI] [PubMed] [Google Scholar]
  48. Kremer Hovinga J.A., Studt J.D., Demarmels Biasiutti F., et al. Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura. Haematologica. 2004;89(3):320–4. [PubMed] [Google Scholar]
  49. Lämmle B., Kremer Hovinga J.A., Alberio L. Thrombotic thrombocytopenic purpura. J Thromb Haemost. 2005;3(8):1663–75. doi: 10.1111/j.1538-7836.2005.01425.x. [DOI] [PubMed] [Google Scholar]
  50. Laurence J., Mitra D., Steiner M., Staiano-Coico L., Jaffe E. Plasma from patients with idiopathic and human immunodeficiency virusassociated thrombotic thrombocytopenic purpura induces apoptosis in microvascular endothelial cells. Blood. 1996;87(8):3245–54. [PubMed] [Google Scholar]
  51. Levy G.G., Nichols W.C., Lian E.C., et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature. 2001;413(6855):488–94. doi: 10.1038/35097008. [DOI] [PubMed] [Google Scholar]
  52. Lopez J.A., Dong J.F. Cleavage of von Willebrand factor by ADAMTS-13 on endothelial cells. Semin Hematol. 2004;41(1):15–23. doi: 10.1053/j.seminhematol.2003.10.004. [DOI] [PubMed] [Google Scholar]
  53. Mannucci P.M., Canciani M.T., Forza I., Lussana F., Lattuada A., Rossi E. Changes in health and disease of the metalloprotease that cleaves von Willebrand factor. Blood. 2001;98(9):2730–5. doi: 10.1182/blood.V98.9.2730. [DOI] [PubMed] [Google Scholar]
  54. Martin K., Borgel D., Lerolle N., Feys H.B., Trinquart L., Vanhoorelbeke K., Deckmyn H., Legendre P., Diehl J.L., Baruch D. Decreased ADAMTS-13 (A disintegrin-like and metalloprotease with thrombospondin type 1 repeats) is associated with a poor prognosis in sepsis-induced organ failure. Crit Care Med. 2007;35(10):2375–82. doi: 10.1097/01.CCM.0000284508.05247.B3. [DOI] [PubMed] [Google Scholar]
  55. Matsumoto M., Kokame K., Soejima K., et al. Molecular characterization of ADAMTS13 gene mutations in Japanese patients with Upshaw-Schulman syndrome. Blood. 2004;103(4):1305–10. doi: 10.1182/blood-2003-06-1796. [DOI] [PubMed] [Google Scholar]
  56. Matsumoto M., Yagi H., Ishizashi H., Wada H., Fujimura Y. The Japanese experience with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Semin Hematol. 2004;41(1):68–74. doi: 10.1053/j.seminhematol.2003.10.009. [DOI] [PubMed] [Google Scholar]
  57. Moake J.L. Thrombotic microangiopathies. N Engl J Med. 2002;347(8):589–600. doi: 10.1056/NEJMra020528. [DOI] [PubMed] [Google Scholar]
  58. Moake J.L., Chow T.W. Thrombotic thrombocytopenic purpura: understanding a disease no longer rare. Am J Med Sci. 1998;316(2):105–19. doi: 10.1097/00000441-199808000-00006. [DOI] [PubMed] [Google Scholar]
  59. Moake J.L., Rudy C.K., Troll J.H., Weinstein M.J., Colannino N.M., Azocar J., Seder R.H., Hong S.L., Deykin D. Unusually large plasma factor VIII: von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med. 1982;307(23):1432–5. doi: 10.1056/NEJM198212023072306. [DOI] [PubMed] [Google Scholar]
  60. Moore J.C., Hayward C.P., Warkentin T.E., Kelton J.G. Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders. Blood. 2001;98(6):1842–6. doi: 10.1182/blood.V98.6.1842. [DOI] [PubMed] [Google Scholar]
  61. Mori Y., Wada H., Gabazza E.C., Minami N., Nobori T., Shiku H., Yagi H., Ishizashi H., Matsumoto M., Fujimura Y. Predicting response to plasma exchange in patients with thrombotic thrombocytopenic purpura with measurement of vWF-cleaving protease activity. Transfusion. 2002;42(5):572–80. doi: 10.1046/j.1537-2995.2002.00100.x. [DOI] [PubMed] [Google Scholar]
  62. Moschcowitz E. Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc N Y Pathol Soc. 1924;24:21–24. [Google Scholar]
  63. Motto D.G., Chauhan A.K., Zhu G., Homeister J., Lamb C.B., Desch K.C., Zhang W., Tsai H.M., Wagner D.D., Ginsburg D. Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice. J Clin Invest. 2005;115(10):2752–61. doi: 10.1172/JCI26007. [DOI] [PMC free article] [PubMed] [Google Scholar]
  64. Nguyen L., Li X., Duvall D., Terrell D.R., Vesely S.K., George J.N. Twicedaily plasma exchange for patients with refractory thrombotic thrombocytopenic purpura: the experience of the Oklahoma Registry, 1989 through 2006. Transfusion. 2008;48(2):349–57. doi: 10.1111/j.1537-2995.2007.01530.x. [DOI] [PubMed] [Google Scholar]
  65. Ono T., Mimuro J., Madoiwa S., Soejima K., Kashiwakura Y., Ishiwata A., Takano K., Ohmori T., Sakata Y. Severe secondary deficiency of von Willebrand factor-cleaving protease (ADAMTS13) in patients with sepsis-induced disseminated intravascular coagulation: its correlation with development of renal failure. Blood. 2006;107(2):528–34. doi: 10.1182/blood-2005-03-1087. [DOI] [PubMed] [Google Scholar]
  66. Peyvandi F., Ferrari S., Lavoretano S., Canciani M.T., Mannucci P.M. von Willebrand factor cleaving protease (ADAMTS-13) and ADAMTS-13 neutralizing autoantibodies in 100 patients with thrombotic thrombocytopenic purpura. Br J Haematol. 2004;127(4):433–9. doi: 10.1111/j.1365-2141.2004.05217.x. [DOI] [PubMed] [Google Scholar]
  67. Peyvandi F., Lavoretano S., Palla R., et al. ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. Haematologica. 2008;93(2):232–9. doi: 10.3324/haematol.11739. [DOI] [PubMed] [Google Scholar]
  68. Pimanda J.E., Maekawa A., Wind T., Paxton J., Chesterman C.N., Hogg P.J. Congenital thrombotic thrombocytopenic purpura in association with a mutation in the second CUB domain of ADAMTS13. Blood. 2004;103(2):627–9. doi: 10.1182/blood-2003-04-1346. [DOI] [PubMed] [Google Scholar]
  69. Plaimauer B., Fuhrmann J., Mohr G., Wernhart W., Bruno K., Ferrari S., Konetschny C., Antoine G., Rieger M., Scheiflinger F. Modulation of ADAMTS13 secretion and specific activity by a combination of common amino acid polymorphisms and a missense mutation. Blood. 2006;107(1):118–25. doi: 10.1182/blood-2005-06-2482. [DOI] [PubMed] [Google Scholar]
  70. Remuzzi G., Galbusera M., Noris M., et al. von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Blood. 2002;100(3):778–85. doi: 10.1182/blood-2001-12-0166. [DOI] [PubMed] [Google Scholar]
  71. Rock G.A. Management of thrombotic thrombocytopenic purpura. Br J Haematol. 2000;109(3):496–507. doi: 10.1046/j.1365-2141.2000.01941.x. [DOI] [PubMed] [Google Scholar]
  72. Rock G.A., Shumak K.H., Buskard N.A., Blanchette V.S., Kelton J.G., Nair R.C., Spasoff R.A. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med. 1991;325(6):393–7. doi: 10.1056/NEJM199108083250604. [DOI] [PubMed] [Google Scholar]
  73. Rüfer A., Brodmann D., Gregor M., Kremer Hovinga J.A., Lämmle B., Wuillemin W.A. Rituximab for acute plasma-refractory thrombotic thrombocytopenic purpura. A case report and concise review of the literature. Swiss Med Wkly. 2007;137(37–38):518–24. doi: 10.4414/smw.2007.11908. [DOI] [PubMed] [Google Scholar]
  74. Ruggenenti P., Remuzzi G. The pathophysiology and management of thrombotic thrombocytopenic purpura. Eur J Haematol. 1996;56(4):191–207. doi: 10.1111/j.1600-0609.1996.tb01930.x. [DOI] [PubMed] [Google Scholar]
  75. Schneppenheim R., Kremer Hovinga J.A., Becker T., et al. A common origin of the 4143insA ADAMTS13 mutation. Thromb Haemost. 2006;96(1):3–6. doi: 10.1160/TH05-12-0817. [DOI] [PubMed] [Google Scholar]
  76. Schneppenheim R., Budde U., Hassenpflug W., Obser T. Severe ADAMTS-13 deficiency in childhood. Semin Hematol. 2004;41(1):83–9. doi: 10.1053/j.seminhematol.2003.10.007. [DOI] [PubMed] [Google Scholar]
  77. Schneppenheim R., Budde U., Oyen F., et al. Von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP. Blood. 2003;101(5):1845–50. doi: 10.1182/blood-2002-08-2399. [DOI] [PubMed] [Google Scholar]
  78. Schultz D.R., Arnold P.I., Jy W., Valant P.A., Gruber J., Ahn Y.S., Mao F.W., Mao W.W., Horstman L.L. Anti-CD36 autoantibodies in thrombotic thrombocytopenic purpura and other thrombotic disorders: identification of an 85 kD form of CD36 as a target antigen. Br J Haematol. 1998;103(3):849–57. doi: 10.1046/j.1365-2141.1998.01070.x. [DOI] [PubMed] [Google Scholar]
  79. Scully M., Cohen H., Cavenagh J., Benjamin S., Starke R., Killick S., Mackie I., Machin S.J. Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS-13. Br J Haematol. 2007;136(3):451–61. doi: 10.1111/j.1365-2141.2006.06448.x. [DOI] [PubMed] [Google Scholar]
  80. Shumak K.H., Rock G.A., Nair R.C. Late relapses in patients successfully treated for thrombotic thrombocytopenic purpura. Ann Intern Med. 1995;122(8):569–72. doi: 10.7326/0003-4819-122-8-199504150-00002. [DOI] [PubMed] [Google Scholar]
  81. Soejima K., Mimura N., Hirashima M., Maeda H., Hamamoto T., Nakagaki T., Nozaki C. A novel human metalloprotease synthesized in the liver and secreted into the blood: possibly, the von Willebrand factor-cleaving protease? J Biochem (Tokyo) 2001;130(4):475–80. doi: 10.1093/oxfordjournals.jbchem.a003009. [DOI] [PubMed] [Google Scholar]
  82. Studt J.D., Kremer Hovinga J.A., Antoine G., Hermann M., Rieger M., Scheiflinger F., Lämmle B. Fatal congenital thrombotic thrombocytopenic purpura with apparent ADAMTS13 inhibitor: in vitro inhibition of ADAMTS13 activity by hemoglobin. Blood. 2005;105(2):542–4. doi: 10.1182/blood-2004-06-2096. [DOI] [PubMed] [Google Scholar]
  83. Studt J.D., Kremer Hovinga J.A., Alberio L., Bianchi V., Lämmle B. Von Willebrand factor-cleaving protease (ADAMTS-13) activity in thrombotic microangiopathies: diagnostic experience 2001/2002 of a single research laboratory. Swiss Med Wkly. 2003;133(23–24):325–32. doi: 10.4414/smw.2003.10242. [DOI] [PubMed] [Google Scholar]
  84. Swisher K.K., Doan J.T., Vesely S.K., Kwaan H.C., Kim B., Lämmle B., Kremer Hovinga J.A., George J.N. Pancreatitis preceding acute episodes of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: report of five patients with a systematic review of published reports. Haematologica. 2007;92(7):936–43. doi: 10.3324/haematol.10963. [DOI] [PubMed] [Google Scholar]
  85. Takahashi H., Tatewaki W., Wada K., Shibata A. Thrombin generation in patients with thrombotic thrombocytopenic purpura. Am J Hematol. 1989;32(4):255–7. doi: 10.1002/ajh.2830320404. [DOI] [PubMed] [Google Scholar]
  86. Tandon N.N., Rock G., Jamieson G.A. Anti-CD36 antibodies in thrombotic thrombocytopenic purpura. Br J Haematol. 1994;88(4):816–25. doi: 10.1111/j.1365-2141.1994.tb05122.x. [DOI] [PubMed] [Google Scholar]
  87. Terrell D.R., Williams L.A., Vesely S.K., Lämmle B., Kremer Hovinga J.A., George J.N. The incidence of thrombotic thrombocytopenic purpurahemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost. 2005;3(7):1432–6. doi: 10.1111/j.1538-7836.2005.01436.x. [DOI] [PubMed] [Google Scholar]
  88. Tsai H.M., Chandler W.L., Sarode R., Hoffman R., Jelacic S., Habeeb R.L., Watkins S.L., Wong C.S., Williams G.D., Tarr P.I. von Willebrand factor and von Willebrand factor-cleaving metalloprotease activity in Escherichia coli O157: H7-associated hemolytic uremic syndrome. Pediatr Res. 2001;49(5):653–9. doi: 10.1203/00006450-200105000-00008. [DOI] [PubMed] [Google Scholar]
  89. Tsai H.M., Rice L., Sarode R., Chow T.W., Moake J.L. Antibody inhibitors to von Willebrand factor metalloproteinase and increased binding of von Willebrand factor to platelets in ticlopidine-associated thrombotic thrombocytopenic purpura. Ann Intern Med. 2000;132(10):794–9. doi: 10.7326/0003-4819-132-10-200005160-00005. [DOI] [PMC free article] [PubMed] [Google Scholar]
  90. Tsai H.M., Lian E.C. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med. 1998;339(22):1585–94. doi: 10.1056/NEJM199811263392203. [DOI] [PMC free article] [PubMed] [Google Scholar]
  91. Tsai H.M. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood. 1996;87(10):4235–44. [PubMed] [Google Scholar]
  92. Uchida T., Wada H., Mizutani M., et al. Identification of novel mutations in ADAMTS13 in an adult patient with congenital thrombotic thrombocytopenic purpura. Blood. 2004;104(7):2081–3. doi: 10.1182/blood-2004-02-0715. [DOI] [PubMed] [Google Scholar]
  93. Upshaw J.D. Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia. N Engl J Med. 1978;298(24):1350–2. doi: 10.1056/NEJM197806152982407. [DOI] [PubMed] [Google Scholar]
  94. Vesely S.K., George J.N., Lämmle B., Studt J.D., Alberio L., El-Harake M.A., Raskob G.E. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood. 2003;102:60–68. doi: 10.1182/blood-2003-01-0193. [DOI] [PubMed] [Google Scholar]
  95. Veyradier A., Obert B., Haddad E., et al. Severe deficiency of the specific von Willebrand factor-cleaving protease (ADAMTS 13) activity in a subgroup of children with atypical hemolytic uremic syndrome. J Pediatr. 2003;142(3):310–7. doi: 10.1067/mpd.2003.79. [DOI] [PubMed] [Google Scholar]
  96. Veyradier A., Obert B., Houllier A., Meyer D., Girma J.P. Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases. Blood. 2001;98(6):1765–72. doi: 10.1182/blood.V98.6.1765. [DOI] [PubMed] [Google Scholar]
  97. Warwicker P., Goodship T.H., Donne R.L., Pirson Y., Nicholls A., Ward R.M., Turnpenny P., Goodship J.A. Genetic studies into inherited and sporadic hemolytic uremic syndrome. Kidney Int. 1998;53(4):836–44. doi: 10.1111/j.1523-1755.1998.00824.x. [DOI] [PubMed] [Google Scholar]
  98. Zheng X.L., Kaufman R.M., Goodnough L.T., Sadler J.E. Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood. 2004;103(11):4043–9. doi: 10.1182/blood-2003-11-4035. [DOI] [PMC free article] [PubMed] [Google Scholar]
  99. Zheng X., Chung D., Takayama T.K., Majerus E.M., Sadler J.E., Fujikawa K. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem. 2001;276(44):41059–63. doi: 10.1074/jbc.C100515200. [DOI] [PubMed] [Google Scholar]

Literatur

  1. Agatisa P.K., Ness R.B., Roberts J.M., et al. Impairment of endothelial function in women with a history of preeclampsia: an indicator of cardiovascular risk. Am J Physiol Heart Circ Physiol. 2004;286:H1389–1393. doi: 10.1152/ajpheart.00298.2003. [DOI] [PubMed] [Google Scholar]
  2. Baxter J.K., Weinstein L. HELLP syndrome: The state of the art. Obstet Gynecol Surv. 2004;59:838–845. doi: 10.1097/01.ogx.0000146948.19308.c5. [DOI] [PubMed] [Google Scholar]
  3. Benyo D.F., Smarason A., Redman C.W., et al. Expression of inflammatory cytokines in placentas from women with preeclampsia. J Clin Endocrinol Metab. 2001;86:2505–2512. doi: 10.1210/jc.86.6.2505. [DOI] [PubMed] [Google Scholar]
  4. Boehlen F. Thrombocytopenie in pregnancy. Haemostaseologie. 2006;1:72–74. [PubMed] [Google Scholar]
  5. Detti L., Mecaci F., Piccioli A., et al. Postpartum heparin therapy for patients with HELLP syndrome is associated with significant hemorrhagic complications. J Perinat. 2005;25:236–240. doi: 10.1038/sj.jp.7211265. [DOI] [PubMed] [Google Scholar]
  6. Dessole S., Capobianco G., Virdis P., et al. Hepatic rupture after cesarean section in a patient with HELLP syndrome: a case report and review of the literature. Arch Gynecol Obstet. 2007;276:189–192. doi: 10.1007/s00404-006-0318-9. [DOI] [PubMed] [Google Scholar]
  7. Eckford S.D., MacNab I.L., Turner M.L., et al. Plasmapheresis in the management of HELLP syndrome. J Obstet Gynecol. 1998;18:377–379. doi: 10.1080/01443619867182. [DOI] [PubMed] [Google Scholar]
  8. Eser B., Guven M., Unal A., et al. The role of plasma exchange in HELLP syndrome. Clin Appl Thrombosis/Hemostasis. 2005;11:211–217. doi: 10.1177/107602960501100211. [DOI] [PubMed] [Google Scholar]
  9. Garovic V.D., Hayman S.R. Hypertension in pregnancy: an emerging risk factor for cardiovascular disease. Nat Clin Pract Nephrol. 2007;3(11):613–622. doi: 10.1038/ncpneph0623. [DOI] [PubMed] [Google Scholar]
  10. Harms K., Rath W., Herting E., Kuhn W. Maternal hemolysis, elevated liver enzymes, low platelet count and neonatal outcome. Am J Perinatol. 1995;12:1–6. doi: 10.1055/s-2007-994387. [DOI] [PubMed] [Google Scholar]
  11. Hulstein J.J., van Runnard Heimpel P.J., Franx A., et al. Acute activation of the endothelium results in increased levels of active von Willebrand factor in hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome. J Thromb Haemost. 2006;4:2569–2575. doi: 10.1111/j.1538-7836.2006.02205.x. [DOI] [PubMed] [Google Scholar]
  12. Jacquemyn Y., Jochems L., Duiker E., et al. Long-term renal function after HELLP syndrome. Gynecol Obstet Invest. 2004;57:117–120. doi: 10.1159/000075942. [DOI] [PubMed] [Google Scholar]
  13. Magann E.F., Martin J.W. Twelve steps to optimal management of HELLP syndrome. Cin Obstet Gynecol. 1999;42:532–543. doi: 10.1097/00003081-199909000-00009. [DOI] [PubMed] [Google Scholar]
  14. Martin J.W., May W.L., Magann E.F., et al. Early risk assessment of severe preeclampsia: admission battery of symptoms and laboratory tests to predict likelihood of subsequent significant maternal morbidity. Am J Obstet Gynecol. 1999;180:1407–1414. doi: 10.1016/S0002-9378(99)70026-8. [DOI] [PubMed] [Google Scholar]
  15. Martin J.W., Rose C.H., Briery C. Understanding and managing HELLP syndrome: the integral role of aggressive glucocorticoids for mother and child. Am J Obstet Gynecol. 2006;195:914–934. doi: 10.1016/j.ajog.2005.08.044. [DOI] [PubMed] [Google Scholar]
  16. Matahaba P, Moodley J (2006) Corticosteroids for HELLP syndrome in pregnancy (Review). The Cochrane Library Issue 2 [DOI] [PubMed]
  17. Rath W., Loos H., Kuhn W. Das HELLP-Syndrom. Zentralbl Gynäkol. 1994;116:195–205. [PubMed] [Google Scholar]
  18. Rath W., Wieding J.U., Kuhn W. Erkenntnisse über hämostaseologische Veränderungen bei Gestose und HELLP-Syndrom für die klinische Praxis. Geburth Frauenheilk. 1991;51:741–746. doi: 10.1055/s-2007-1023825. [DOI] [PubMed] [Google Scholar]
  19. Rath W., Faridi A., Dudenhausen J.W. HELLP syndrome. J Perinat Med. 2000;28:249–260. doi: 10.1515/JPM.2000.033. [DOI] [PubMed] [Google Scholar]
  20. Rath W. Hypertensive Schwangerschaftserkrankungen. In: Rath W., Friese K., editors. Erkrankungen in der Schwangerschaft. Stuttgart: Thieme; 2005. pp. 73–97. [Google Scholar]
  21. Rath W., Fischer T., Klockenbusch W., et al. Diagnostik und Therapie hypertensiver Schwangerschaftserkrankungen. AWMF. 2007;015/018:2. [Google Scholar]
  22. Sibai B.M., El-Nazar A., Gonzalez-Ruiz A. Severe preeclampsia-eclampsia in young primigravid women. Subsequent pregnancy outcome and remote prognosis. Am J Obstet Gynecol. 1986;155:1011–1016. doi: 10.1016/0002-9378(86)90336-4. [DOI] [PubMed] [Google Scholar]
  23. Sibai B.M., Ramadan M.K., Usta J., et al. Maternal morbidity and mortality in 442 patients with HELLP syndrome. Am J Obstet Gynecol. 1993;169:1000–1006. doi: 10.1016/0002-9378(93)90043-i. [DOI] [PubMed] [Google Scholar]
  24. Visser W., Wallenburg H.C.S. Temporising management of severe preeclampsia with and without HELLP syndrome. BJOG. 1995;102:111–120. doi: 10.1111/j.1471-0528.1995.tb09062.x. [DOI] [PubMed] [Google Scholar]

Literatur

  1. Abraham E., Reinhart K., Svoboda P., et al. Assessment of the safety of recombinant tissue factor pathway inhibitor in patients with severe sepsis: a multicenter, randomized, placebo-controlled, singleblind, dose escalation study. Crit Care Med. 2001;29:2081–2089. doi: 10.1097/00003246-200111000-00007. [DOI] [PubMed] [Google Scholar]
  2. Abraham E., Reinhart K., Opal S., et al. Efficacy and safety of tifacogin (recombinant tissue factor pathway inhibitor) in severe sepsis: a randomized controlled trial. JAMA. 2003;290:238–247. doi: 10.1001/jama.290.2.238. [DOI] [PubMed] [Google Scholar]
  3. Angstwurm M.W.A., Dempfle C.E., Spannagl M. New disseminated intravascular coagulation score: a useful tool to predict mortality in comparison with acute physiology and chronic health evaluation II and logistic organ dysfunction scores. Crit Care Med. 2006;34:314–320. doi: 10.1097/01.CCM.0000196832.27501.B2. [DOI] [PubMed] [Google Scholar]
  4. Avvisati G., ten Cate J.W., Büller H.R., et al. Tranexamic acid for control of haemorrhage in acute promyelocytic leukamia. Lancet. 1989;2:122–124. doi: 10.1016/S0140-6736(89)90181-5. [DOI] [PubMed] [Google Scholar]
  5. Bakhtiari K., Meijers J.C., de Jonge E., et al. Prospective validation of the International Society of Thrombosis and Haemostasis scoring system for disseminated intravascular coagulation. Crit Care Med. 2004;32:2416–2421. doi: 10.1097/01.CCM.0000147769.07699.E3. [DOI] [PubMed] [Google Scholar]
  6. BarbuiT F. A. Disseminated intravascular coagulation in acute leukaemia. Semin Thromb Hemost. 2001;27:593–604. doi: 10.1055/s-2001-18865. [DOI] [PubMed] [Google Scholar]
  7. Bernard G.R., Vincent J.L., Laterre P.F., Recombinant human protein C Worldwide Evaluation in Severe Sepsis PROWESS) study group et al. Efficacy and safety of recombinant human activated protein C for severe sepsis. N Engl J Med. 2001;344:699–709. doi: 10.1056/NEJM200103083441001. [DOI] [PubMed] [Google Scholar]
  8. Bone R.C. Gram-positive organisms and sepsis. Arch Intern Med. 1994;154:26–34. doi: 10.1001/archinte.154.1.26. [DOI] [PubMed] [Google Scholar]
  9. Carr J.M., McKinney M., McDonagh J. Diagnosis of disseminated intravascular coagulation: role of D-Dimer. Am J Clin Pathol. 1989;91:280–287. doi: 10.1093/ajcp/91.3.280. [DOI] [PubMed] [Google Scholar]
  10. Colman R.W., Rubin R.N. Disseminated intravascular coagulation due to malignancy. Semin Oncol. 1990;17:172–186. [PubMed] [Google Scholar]
  11. Corrigan J.J., Jr Heparin therapy in bacterial septicemia. J Pediatr. 1977;91:695–700. doi: 10.1016/S0022-3476(77)81017-2. [DOI] [PubMed] [Google Scholar]
  12. De Pont A.C., Bakhtiari K., Hutten B.A., et al. Recombinant human activated protein C resets thrombin generation in patients with severe sepsis: A case control study. Crit Care. 2005;9:R490–R497. doi: 10.1186/cc3774. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Dhainaut J.F., Yan S.B., Joyce D.E., et al. Treatment effects of drotrecogin alfa (activated) in patients with severe sepsis with or without overt disseminated intravascular coagulation. J Thromb Haemost. 2004;2:1924–1933. doi: 10.1111/j.1538-7836.2004.00955.x. [DOI] [PubMed] [Google Scholar]
  14. Dhainaut J.F., Shorr A.F., Macias W.L., et al. Dynamic evolution of coagulopathy in the first day of severe sepsis: relationship with mortality and organ failure. Crit Care Med. 2005;33:341–348. doi: 10.1097/01.CCM.0000153520.31562.48. [DOI] [PubMed] [Google Scholar]
  15. Dempfle C.E. The use of soluble fibrin in evaluating the acute and chronic hypercoagulable state. Thromb Haemost. 1999;82:673–683. [PubMed] [Google Scholar]
  16. du Toit H., Coetzee A.R., Chalton D.O. Heparin treatment in thrombin-induced disseminated intravascular coagulation in the baboon. Crit Care Med. 1991;19:1195–1200. doi: 10.1097/00003246-199109000-00017. [DOI] [PubMed] [Google Scholar]
  17. Feinstein D.I. Diagnosis and management of disseminated intravascular coagulation: the role of heparin therapy. Blood. 1982;60:284–287. [PubMed] [Google Scholar]
  18. Gando S., Sawamura A., Hayakawa M., Hoshino H., Kubota N., Oshiro A. First day dynamic changes in antithrombin III activity after supplementation have a predictive value in critically ill patients. Am J Hematol. 2006;81:907–14. doi: 10.1002/ajh.20696. [DOI] [PubMed] [Google Scholar]
  19. Gando S., Nakanisihi Y., Tedo I. Cytokines and plasminogen activator inhibitor-1 in posttrauma disseminated intravascular coagulation: relationship to multiple organ dysfunction syndrome. Crit Care Med. 1995;23:1835–1842. doi: 10.1097/00003246-199511000-00009. [DOI] [PubMed] [Google Scholar]
  20. Kienast J., Juers M., Wiedermann C.J., et al. Treatment effects of high-dose antithrombin with concomitant heparin in patients with severe sepsis with or without disseminated intravascular coagulation. J Thromb Haemost. 2006;4:90–97. doi: 10.1111/j.1538-7836.2005.01697.x. [DOI] [PubMed] [Google Scholar]
  21. Kinasewitz G.T., Zein J.G., Lee G.L. Prognostic value of a simple evolving disseminated intravascular coagulation score in patients with severe sepsis. Crit Care Med. 2005;33:2214–2221. doi: 10.1097/01.CCM.0000181296.53204.DE. [DOI] [PubMed] [Google Scholar]
  22. Kobayshi N., Maekawa T., Takada M., et al. Criteria for diagnosis of DIC based on the analysis of clinical and laboratory findings in 345 DIC patients collected by the research committee on DIC in Japan. Bibl Haematol. 1987;49:848–852. doi: 10.1159/000408467. [DOI] [PubMed] [Google Scholar]
  23. Levi M., van der Poll T., ten Cate H., et al. The cytokine-mediated imbalance between coagulant and anticoagulant mechanisms in sepsis and endotoxaemia. Eur J Clin Invest. 1997;27:3–9. doi: 10.1046/j.1365-2362.1997.570614.x. [DOI] [PubMed] [Google Scholar]
  24. Levi M., ten Cate H. Disseminated intravascular coagulation. N Engl J Med. 1999;341:586–592. doi: 10.1056/NEJM199908193410807. [DOI] [PubMed] [Google Scholar]
  25. Levi M., de Jonge E., van der Poll T. Rationale for restoration of physiological anticoagulant pathways in patients with sepsis and disseminated intravascular coagulation. Crit Care Med. 2001;29:S90–S94. doi: 10.1097/00003246-200107001-00028. [DOI] [PubMed] [Google Scholar]
  26. Levi M. The imbalance between tissue factor and tissue factor pathway inhibitor in sepsis. Crit Care Med. 2002;30:1914–1915. doi: 10.1097/00003246-200208000-00046. [DOI] [PubMed] [Google Scholar]
  27. Levi M., Peters M., Buller H.R. Efficacy and safety of recombinant factor VIIa for the treatment of severe bleeding: a systematic review. Crit Care Med. 2005;33:883–890. doi: 10.1097/01.CCM.0000159087.85970.38. [DOI] [PubMed] [Google Scholar]
  28. Levi M., van der Poll T., ten Cate H. Tissue factor in infection and severe inflammation. Semin Thromb Hemost. 2006;32:33–39. doi: 10.1055/s-2006-933338. [DOI] [PubMed] [Google Scholar]
  29. Levi M. Disseminated intravascular coagulation. Crit Care Med. 2007;35:2191–2195. doi: 10.1097/01.CCM.0000281468.94108.4B. [DOI] [PubMed] [Google Scholar]
  30. Mannucci P.M., Levi M. Prevention and treatment of major blood loss. N Engl J Med. 2007;356:2301–2311. doi: 10.1056/NEJMra067742. [DOI] [PubMed] [Google Scholar]
  31. McCarron B.I., Marder V.J., Francis C.W. Reactivity of soluble fibrin assays with plasmic degradation products of fibrin and in patients receiving fibrinolytic therapy. Thromb Haemost. 1999;82:1722–1729. [PubMed] [Google Scholar]
  32. Ontachi Y., Asakura H., Arahata M., et al. Effect of combined therapy of danaparoid sodium and tranexamic acid on chronic disseminated intravascular coagulation associated with abdominal aortic aneurysm. Circ J. 2005;69:1150–1153. doi: 10.1253/circj.69.1150. [DOI] [PubMed] [Google Scholar]
  33. Pernerstorfer T., Hollenstein U., Hansen J., et al. Heparin blunts endotoxin-induced coagulation activation. Circulation. 1999;100:2485–2490. doi: 10.1161/01.cir.100.25.2485. [DOI] [PubMed] [Google Scholar]
  34. Slofstra S.H., vant Veer C., Buurman W.A., et al. Low molecular weight heparin attenuates multiple organ failure in a murine model of disseminated intravascular coagulation. Crit Care Med. 2005;33:1455–1457. doi: 10.1097/01.CCM.0000166370.94927.B6. [DOI] [PubMed] [Google Scholar]
  35. Taylor F.B.J., Chang A., Esmon C.T., et al. Protein C prevents the coagulopathic and lethal effects of Escherichia coli infusion in the baboon. J Clin Invest. 1987;79:918–925. doi: 10.1172/JCI112902. [DOI] [PMC free article] [PubMed] [Google Scholar]
  36. Taylor F.B.J., Chang A., Ruf W., et al. Lethal E. coli septic shock is prevented by blocking tissue factor with monoclonal antibody. Circ Shock. 1991;33:127–134. [PubMed] [Google Scholar]
  37. Ten Cate H., Timmerman J.J., Levi M. The pathophysiology of disseminated intravascular coagulation. Thromb Haemost. 1999;82:713–717. [PubMed] [Google Scholar]
  38. Toh C.H., Dennis M. Disseminated intravascular coagulation: old disease, new hope. BMJ. 2005;327:974–977. doi: 10.1136/bmj.327.7421.974. [DOI] [PMC free article] [PubMed] [Google Scholar]
  39. Warren B.L., Eid A., Singer P., et al. Caring for the critically ill patient: high-dose antithrombin III in severe sepsis. A randomized controlled trial. JAMA. 2001;286:1869–1878. doi: 10.1001/jama.286.15.1869. [DOI] [PubMed] [Google Scholar]
  40. Weiner C.P. The obstetric patient and disseminated intravascular coagulation. Clin Perinatol. 1986;13:705–717. [PubMed] [Google Scholar]

Literatur

  1. Anagrelide Study Group Anagrelide, a therapy for thrombocythemic states. Experience in 577 patients. Am J Med. 1992;92:69–76. doi: 10.1016/0002-9343(92)90017-6. [DOI] [PubMed] [Google Scholar]
  2. Apperley J., Gardembas M., Melo J., et al. Response to imatinib mesylate in patients with chronic myeloproliferative diseases with rearrangements of the platelet-derived growth factor receptor beta. N Engl J Med. 2002;347:481–487. doi: 10.1056/NEJMoa020150. [DOI] [PubMed] [Google Scholar]
  3. Bench A., Pahl H. Chromosomal and molecular markers in myeloproliferative disorders. Semin Hematol. 2005;42:196–205. doi: 10.1053/j.seminhematol.2005.08.001. [DOI] [PubMed] [Google Scholar]
  4. Berk P.D., Goldberg J.D., Silverstein M.N., et al. Increased incidence of acute leukemia in polycythemia vera associated with chlorambucil therapy. N Engl J Med. 1981;304:441–447. doi: 10.1056/NEJM198102193040801. [DOI] [PubMed] [Google Scholar]
  5. Campbell P., Scott L., Buck G., et al. Definition of subtypes of essential thrombocythemia and relation to polycythemia vera based on JAK2V617F mutation ststus: a prospective study. Lancet. 2005;366:1945–1953. doi: 10.1016/S0140-6736(05)67785-9. [DOI] [PubMed] [Google Scholar]
  6. Cervantes F., Barosi G., Demory J., et al. Myelofibrosis with myeloid metaplasia in young individuals: disease characteristics, prognostic factors and identification of risk Groups. Brit J Haematol. 1998;102:684–690. doi: 10.1046/j.1365-2141.1998.00833.x. [DOI] [PubMed] [Google Scholar]
  7. Cortelazzo S., Viero P., Bellavita P., et al. Hydroxyurea for patients with essential thrombocythemia and a high risk of thrombosis. N Engl J Med. 1995;332:1132–1136. doi: 10.1056/NEJM199504273321704. [DOI] [PubMed] [Google Scholar]
  8. Cross N.C., Reiter A. Tyrosine kinase fusion genes in chronic myeloproliferative diseases. Leukemia. 2002;16:1207–1212. doi: 10.1038/sj.leu.2402556. [DOI] [PubMed] [Google Scholar]
  9. Finazzi G., Ruggeri M., Rodeghiero F., et al. Second malignancies in patients with essential thrombocythemia treated with busulphan and hydroxyurea: long-term follow up of a randomized trial. Brit J Haematol. 2000;110:577–583. doi: 10.1046/j.1365-2141.2000.02188.x. [DOI] [PubMed] [Google Scholar]
  10. Gotlib J., Cools J., Malone J., et al. The FIP1L1-PDGFRα fusion tyrosine kinase in hypereosinophilic syndrome and chronic eosinophilc leukemia: implications for diagnosis, classification and management. Blood. 2004;103:2879–2891. doi: 10.1182/blood-2003-06-1824. [DOI] [PubMed] [Google Scholar]
  11. Gruppo Italiano Studio Polycythemia vera The natural history of 1213 patients followed for 20 years. Ann Intern Med. 1995;123:656–664. doi: 10.7326/0003-4819-123-9-199511010-00003. [DOI] [PubMed] [Google Scholar]
  12. Harrison C.N., Campbell P.J., Buck G., et al. United Kingdom Medical Research Council Primary Thrombocythemia 1 Study. Hydroxyurea compared with anagrelide in high-risk essential thrombocythemia. N Engl J Med. 2005;353:33–45. doi: 10.1056/NEJMoa043800. [DOI] [PubMed] [Google Scholar]
  13. James C., Ugo V., Couédic J., et al. A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera. Nature. 2005;434:1144–1148. doi: 10.1038/nature03546. [DOI] [PubMed] [Google Scholar]
  14. Jones A., Kreil S., Zoi K., et al. Widespread occurrence of the JAK2V617F mutation in chronic myeloproliferative disorders. Blood. 2005;106:2162–2168. doi: 10.1182/blood-2005-03-1320. [DOI] [PubMed] [Google Scholar]
  15. Klippel S., Strunck S., Temerinac S., et al. Quantification of PRV-1 mRNA distinguishes polycythemia vera from secondary erythrocytosis. Blood. 2003;102:3569–3574. doi: 10.1182/blood-2003-03-0919. [DOI] [PubMed] [Google Scholar]
  16. Landolfi R., Marchioli R., Kutti J., et al. Efficacy and safety of low dose aspirin in polycythemia vera. N Engl J Med. 2004;350:114–124. doi: 10.1056/NEJMoa035572. [DOI] [PubMed] [Google Scholar]
  17. Lengfelder E., Griesshammer M., Hehlmann R. Interferon-alpha in the treatment of essential thrombocythemia. Leukemia and Lymphoma. 1997;22(Suppl 1):135–142. doi: 10.3109/10428199609074371. [DOI] [PubMed] [Google Scholar]
  18. Lengfelder E., Hochhaus A., Kronawitter U., et al. Should a platelet limit of 600 *10 9/l be used as a diagnostic criterion in essential thrombocythemia? An analysis of the natural course including early stages. Brit J Haematol. 1998;100:15–23. doi: 10.1046/j.1365-2141.1998.00529.x. [DOI] [PubMed] [Google Scholar]
  19. Lengfelder E., Berger U., Hehlmann R. Interferon treatment of polycythemia vera. Ann Hematol. 2000;79:103–109. doi: 10.1007/s002770050563. [DOI] [PubMed] [Google Scholar]
  20. Najean Y., Dresch C., Rain J.D. The very-long-term course of polycythemia: a complement to the previously published data of the polycythemia vera study group. Brit J Haematol. 1993;86:233–235. doi: 10.1111/j.1365-2141.1994.tb03289.x. [DOI] [PubMed] [Google Scholar]
  21. Tartaglia A., Goldberg J.D., Berk P.D., Wasserman L.R. Adverse effects of antiaggregating platelet therapy in the treatment of polycythemia vera. Semin Hematol. 1986;23:172–176. [PubMed] [Google Scholar]
  22. Tefferi A., Thiele J., Orazio A., et al. Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis: recommendation from an ad hoc international expert panel. Blood. 2007;110:1092–1097. doi: 10.1182/blood-2007-04-083501. [DOI] [PubMed] [Google Scholar]
  23. Tefferi A. Myelofibrosis with myeloid metaplasia. N Engl J Med. 2000;342:1255–1265. doi: 10.1056/NEJM200004273421706. [DOI] [PubMed] [Google Scholar]
  24. Thiele J. Prefibrotic and classical chronic idiopathic myelofibrosis or myelofibrosis with myeloid metaplasia/agnogenic myeloid metaplasia. In: Michiels J.J., Kvasnicka M.M., Thiele J., editors. Myeloproliferative disorders. Bad Honnef: Grunwald; 2005. pp. 62–83. [Google Scholar]

Literatur

  1. Alpert L.I. Veno-occlusive disease of the liver associated with oral contraceptives: case report and review of literature. Hum Pathol. 1976;7:709–18. doi: 10.1016/S0046-8177(76)80082-2. [DOI] [PubMed] [Google Scholar]
  2. Annaloro C., Robbiolo L., Pozzoli E., et al. Four-year survival after trans-jugular intrahepatic porto-systemic shunt für veno-occlusive disease following autologous bane marrow transplantation. Leuk Lymphoma. 2004;45:1485–1487. doi: 10.1080/10428190410001663626. [DOI] [PubMed] [Google Scholar]
  3. Attal M., Huguet F., Rubie H., et al. Prevention of hepatic veno-occlusive disease after bane marrow transplantation by continuous infusion of low-dose heparin: a prospective, randomized trial. Blood. 1992;79:2834–2840. [PubMed] [Google Scholar]
  4. Ayash L.J., Hunt M., Antman K., et al. Hepatic venoocclusive disease in autologous bane marrow transplantation of solid tumors and lymphomas. J Clin Oncol. 1990;8:1699–1706. doi: 10.1200/JCO.1990.8.10.1699. [DOI] [PubMed] [Google Scholar]
  5. Azoulay D., Castaing D., Lemoine A., et al. Transjugular intrahepatic portosystemic shunt (TIPS) for severe veno-occlusive disease of the liver following bone marrow transplantation. Bone Marrow Transplant. 2000;25:987–92. doi: 10.1038/sj.bmt.1702386. [DOI] [PubMed] [Google Scholar]
  6. Bacher P., Kindel G., Walenga J.M., et al. Modulation of endothelial and platelet function by a polydeoxyribonucleotide derived drug “efibrotide„. A dual mechanism in the control of vascular pathology. Thromb Res. 1993;70:343–8. doi: 10.1016/0049-3848(93)90107-Y. [DOI] [PubMed] [Google Scholar]
  7. Barkholt L., Remberger M., Hassan Z., et al. A prospective randomized study using N-acetyl-L-cysteine for early liver toxicity after allogeneic hematopoietic stem cell transplantation. Bone Marrow Transplant. 2008;41:785–90. doi: 10.1038/sj.bmt.1705969. [DOI] [PubMed] [Google Scholar]
  8. Bearman S.I., Lee J.L., Baron A.E., et al. Treatment of hepatic venoocclusive disease with recombinant human tissue plasminogen activator and heparin in 42 Marrow transplant patients. Blood. 1997;89:1501–1506. [PubMed] [Google Scholar]
  9. Bearman S.I., Shuhart M.C., Hinds M.S., et al. Recombinant human tissue plasminogen activator für the treatment of established severe venoocclusive disease of the liver after bane marrow transplantation. Blood. 1992;80:2458–2462. [PubMed] [Google Scholar]
  10. Berk P.D., Popper H., Krueger G.R., et al. Veno-occlusive disease of the liver after allogenic bane marrow transplantation: possible association with graft-versus-host disease. Ann Intern Med. 1979;90:158–164. doi: 10.7326/0003-4819-90-2-158. [DOI] [PubMed] [Google Scholar]
  11. Bianchi G., Barone D., Lanzarotti E., et al. Defibrotide, a single-stranded polydeoxyribonucleotide acting as an adenosine receptor agonist. Eur J Pharmacol. 1993;238:327–334. doi: 10.1016/0014-2999(93)90864-E. [DOI] [PubMed] [Google Scholar]
  12. Bras G., Jelliffe D.B., Stuart K.L. Veno-occlusive disease of liver with nonportal type of cirrhosis, occurring in Jamaica. AMA Arch Pathol. 1954;57:285–300. [PubMed] [Google Scholar]
  13. Bulley S.R., Strahm B., Doyle J., et al. Defibrotide fort he treatment of hepatic veno-occlusive disease in children. Pediatr Blood Cancer. 2006;48:700–4. doi: 10.1002/pbc.20934. [DOI] [PubMed] [Google Scholar]
  14. Carreras E. Veno-occlusive disease of the liver after hematopoietic cell transplantation. Eur J Haematol. 2000;64:281–291. doi: 10.1034/j.1600-0609.2000.9r200.x. [DOI] [PubMed] [Google Scholar]
  15. Carreras E., Bertz H., Arcese W., et al. Incidence and outcome of hepatic veno-occlusive disease after blood or marrow transplantation: a prospective cohort study of the European Group for Blood and Marrow Transplantation. European Group for Blood and Marrow Transplantation Chronic Leukemia Working Party. Blood. 1998;92:3599–3604. [PubMed] [Google Scholar]
  16. Carreras E., Graenena A., Navasa M., et al. On the reliability of clinical criteria for the diagnosis of hepatic veno-occlusive disease. Ann Hematol. 1993;66:77–80. doi: 10.1007/BF01695888. [DOI] [PubMed] [Google Scholar]
  17. Chalandon Y., Roosnek E., Mermillod B., et al. Prevention of venoocclusive disease with defibrotide after allogeneic stern cell transplantation. Biol Blood Marrow Transplant. 2004;10:347–354. doi: 10.1016/j.bbmt.2004.01.002. [DOI] [PubMed] [Google Scholar]
  18. Chopra R., Eaton J.D., Grassi A., et al. Defibrotide for the treatment of hepatic veno-occlusive disease: results of the European compassionate-use study. Br J Haematol. 2000;111:1122–1129. doi: 10.1046/j.1365-2141.2000.02475.x. [DOI] [PubMed] [Google Scholar]
  19. Clift R.A., Buckner C.D., Appelbaum F.R., et al. Allogeneic marrow transplantation in patients with acute myeloid leukemia in first remission: a randomized trial of two irradiation regimens. Blood. 1990;76:1867–71. [PubMed] [Google Scholar]
  20. Corbacioglu S., Greil J., Peters C., et al. Defibrotide in the treatment of children with veno-occlusive disease (VOD): a retrospective multicentre study demonstrates therapeutic efficacy upon early intervention. Bone Marrow Transplant. 2004;33:189–95. doi: 10.1038/sj.bmt.1704329. [DOI] [PubMed] [Google Scholar]
  21. DeLeve L.D., Shulmann H.M., McDonald G.B. Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease) Semin Liver Dis. 2002;22:27–42. doi: 10.1055/s-2002-23204. [DOI] [PubMed] [Google Scholar]
  22. DeLeve L.D., McCuskey R.S., Wang X., et al. Characterization of a reproducible rat model of hepatic veno-occlusive disease. Hepatology. 1999;29:1779–1791. doi: 10.1002/hep.510290615. [DOI] [PubMed] [Google Scholar]
  23. Dignan F., Gujral D., Ethell M., et al. Prophylactic defibrotide in allogeneic stem cell transplantation: minimal morbidity and zero mortality from veno-occlusive disease. Bone Marrow Transplant. 2007;40:79–82. doi: 10.1038/sj.bmt.1705696. [DOI] [PubMed] [Google Scholar]
  24. Dulley F.L., Kanfer E.J., Appelbaum F.R., et al. Venoocclusive disease of the liver after chemoradiotherapy and autologous bone marrow transplantation. Transplantation. 1987;43:870–3. [PubMed] [Google Scholar]
  25. El Mouelhi M., Kauffmann F.C. Sublobular distribution of tranferases and hydrolases associated with glucuronide, sulfate and glutathione conjugation in human liver. Hepatology. 1986;6:450–6. doi: 10.1002/hep.1840060322. [DOI] [PubMed] [Google Scholar]
  26. Essell J.H., Schroeder M.T., Harman G.S., et al. Ursodil prophylaxis against hepatic complications of allogeneic bane marrow transplantation. A randomized, double-blind, placebo-controlled trial. Ann Intern Med. 1998;128:975–981. doi: 10.7326/0003-4819-128-12_part_1-199806150-00002. [DOI] [PubMed] [Google Scholar]
  27. Essell J.H., Thompson J.M., Harman G.S., et al. Marked increase in veno-occlusive disease of the liver associated with methotrexate use for graft-versus-host disease prophylaxis in patients receiving busulfan/cyclophosphamide. Blood. 1992;79:2784–2788. [PubMed] [Google Scholar]
  28. Fajardo L.F., Colby T.V. Pathogenesis of veno-occlusive liver disease after radiation. Arch Pathol Lab Med. 1980;104:584–588. [PubMed] [Google Scholar]
  29. Ferra C., de Sanjose S., Gallardo D., et al. IL-6 and IL-8 levels in plasma during hematopoietic progenitor transplantation. Haematologica. 1998;83:1082–7. [PubMed] [Google Scholar]
  30. Fisher D.C., Vredenburgh J.J., Petros W.P., et al. Reduced mortality following bone marrow transplantation fro breast cancer with the addition of peripheral blood progenitor cells is due to a marked reduction in veno-occlusive disease of the liver. Bone marrow transplan. 1998;21:117–22. doi: 10.1038/sj.bmt.1701068. [DOI] [PubMed] [Google Scholar]
  31. Forrest D.L., Thompson K., Dorcas V.G., et al. Low molecular weight heparin for the prevention of hepatic veno-occlusive disease (VOD) after hematopoietic stem cell transplantation: a prospective phase II study. Bone Marrow Transplant. 2003;31:1143–9. doi: 10.1038/sj.bmt.1704087. [DOI] [PMC free article] [PubMed] [Google Scholar]
  32. Fried M.W., Connagan D.G., Sharma S., et al. Transjugular intrahepatic portosystemic shunt for the management of severe venoocclusive disease following bone marrow transplantation. Hepatology. 1996;24:588–91. doi: 10.1002/hep.510240321. [DOI] [PubMed] [Google Scholar]
  33. Goringe A.P., Brown S., O’Callaghan U., et al. Glutamine and vitamin E in the treatment of hepatic veno-occlusive disease following high-dose chemotherapy. Bone Marrow Transplant. 1998;21:829–32. doi: 10.1038/sj.bmt.1701172. [DOI] [PubMed] [Google Scholar]
  34. Hagglund H., Remberger M., Klaesson S., et al. Norethisterone treatment, a major risk-factor tor veno-occlusive disease in the liver after allogeneic bane marrow transplantation. Blood. 1998;92:4568–4572. [PubMed] [Google Scholar]
  35. Hagglund H., Ringden O., Ericzon B.G., et al. Treatment of hepatic venoocclusive disease with recombinant human tissue plasminogen activator or orthotopic liver transplantation after allogeneic bone marrow transplantation. Transplantation. 1996;62:1076–80. doi: 10.1097/00007890-199610270-00009. [DOI] [PubMed] [Google Scholar]
  36. Ho V.T., Linden E., Revta C., et al. Heaptic veno-occlusive disease after hematopoietic stem cell transplantation: Review and update on the use of defobrotide. Semin Thromb Hemost. 2007;33:373–88. doi: 10.1055/s-2007-976173. [DOI] [PubMed] [Google Scholar]
  37. Jones R.J., Lee K.S., Beschorner W.E., et al. Venoocclusive disease of the liver following bane marrow transplantation. Transplantation. 1987;44:778–783. doi: 10.1097/00007890-198712000-00011. [DOI] [PubMed] [Google Scholar]
  38. Leahey A.M., Bunin N.J. Recombinant human tissue plasminogen activator for the treatment of severe hepatic veno-occlusive disease in pediatric bane marrow transplant patients. Bone Marrow Transplant. 1996;17:1101–1104. [PubMed] [Google Scholar]
  39. Lee J.H., Lee K.H., Lee J.H., et al. Plasminogen activator inhibitor-1 is an independent diagnostic marker as well as severity predictor of hepatic veno-occlusive disease after allogeneic bone marrow transplantation in adults conditioned with busulfan and cyclophosphamide. Br J Haematol. 2002;118:1087–94. doi: 10.1046/j.1365-2141.2002.03748.x. [DOI] [PubMed] [Google Scholar]
  40. MacQuillan G.C., Mutimer D. Fulminant liver failure due to severe veno-occlusive disease after haematopoietic cell transplantation: A depressing experience. Q J Med. 2004;97:581–9. doi: 10.1093/qjmed/hch095. [DOI] [PubMed] [Google Scholar]
  41. Mandel J., Mark E.J., Hales C.A. Pulmonary veno-occlusive disease. Am J Respir Crit Care Med. 2000;162:1964–73. doi: 10.1164/ajrccm.162.5.9912045. [DOI] [PubMed] [Google Scholar]
  42. Marsa-Vila L., Gorin N.C., Laporte J.P., et al. Prophylactic heparin does not prevent liver veno-occlusive disease following autologous bone marrow transplantation. Eur J Haematol. 1991;47:346–54. doi: 10.1111/j.1600-0609.1991.tb01859.x. [DOI] [PubMed] [Google Scholar]
  43. Matute-Bello G., McDonald G.D., Hinds M.S., et al. Association of pulmonary function testing abnormalities and severe veno-occlusive disease of the liver after bone marrow transplantation. Bone Marrow Transplant. 1998;21:1125–30. doi: 10.1038/sj.bmt.1701225. [DOI] [PubMed] [Google Scholar]
  44. McDonald G.B., Hinds M.S., Fisher L.D., et al. Veno-occlusive disease of the liver and multiorgan failure after bane marrow transplantation: a cohort study of 355 patients. Ann Intern Med. 1993;118:255–267. doi: 10.7326/0003-4819-118-4-199302150-00003. [DOI] [PubMed] [Google Scholar]
  45. McDonald G.B., Sharma P., Matthews D.E., et al. Venoocclusive disease of the liver after bane marrow transplantation: diagnosis, incidence, and predisposing factors. Hepatology. 1984;4:116–122. doi: 10.1002/hep.1840040121. [DOI] [PubMed] [Google Scholar]
  46. Merresse V., Hartmann O., Vassal G., et al. Risk factors for hepatic veno-occlusive disease after high-dose busulfan-containing regimens followed by autologous bane marrow transplantation: a study of 136 children. Bone Marrow Transplant. 1992;10:135–141. [PubMed] [Google Scholar]
  47. Morris J.D., Harris R.E., Hashmi R., et al. Antithrombin-111 for the treatment of chemotherapy-induced organ dysfunction following bane marrow transplantation. Bone Marrow Transplant. 1997;20:871–878. doi: 10.1038/sj.bmt.1700985. [DOI] [PubMed] [Google Scholar]
  48. Nattakom T.V., Charlton A., Wilmore D.W. Use of vitamin E and glutamine in the successful treatment of severe veno-occlusive disease following bone marrow transplantation. Nutr Clin Pract. 1995;10:16–8. doi: 10.1177/011542659501000116. [DOI] [PubMed] [Google Scholar]
  49. Nimer S.D., Milewicz A.L., Champlin R.E., et al. Successful treatment of hepatic venoocclusive disease in a bane marrow transplant patient with orthotopic liver transplantation. Transplantation. 1990;49:819–821. doi: 10.1097/00007890-199004000-00036. [DOI] [PubMed] [Google Scholar]
  50. Ohashi K., Tanabe J., Watanabe R., et al. The Japanese multicenter open randomized trial of ursodeoxycholic acid prophylaxis for hepatic veno-occlusive disease after stern cell transplantation. Am J Hematol. 2000;64:32–38. doi: 10.1002/(SICI)1096-8652(200005)64:1<32::AID-AJH6>3.0.CO;2-N. [DOI] [PubMed] [Google Scholar]
  51. Or R., Nagler A., Shpilberg O., et al. Low molecular weight heparin for the prevention of veno-occlusive disease of the liver in bone marrow transplantation patients. Transplantation. 1996;61:1067–71. doi: 10.1097/00007890-199604150-00014. [DOI] [PubMed] [Google Scholar]
  52. Petäjä J., Pitkänen S., Vettenranta K., et al. Serum tumor marker CA 125 is an early and sensitive indicator of veno-occlusive disease in children undergoing bone marrow transplantation. Clin Cancer Res. 2000;6:531–5. [PubMed] [Google Scholar]
  53. Pihusch M., Wegner H., Goehring P., et al. Diagnosis of hepatic veno-occlusive disease by plasminogen activator inhibitor-1 plasma antigen levels: a prospective analysis in 350 allogeneic hematopoietic stem cell recipients. Transplantation. 2005;80:1376–82. doi: 10.1097/01.tp.0000183288.67746.44. [DOI] [PubMed] [Google Scholar]
  54. Rabiller A., Jais X., Hamid A., et al. Occult alveolar haemorrhage in pulmonary veno-occlusive disease. Eur Respir J. 2006;27:108–113. doi: 10.1183/09031936.06.00054105. [DOI] [PubMed] [Google Scholar]
  55. Rapaport A.P., Doyle H.R., Starzl T., et al. Orthotopic liver transplantation for life-threatening veno-occlusive disease of the liver after allogeneic bane marrow transplant. Bone Marrow Transplant. 1991;8:421–424. [PubMed] [Google Scholar]
  56. Richardson P.G., Soiffer R., Antin J.H., et al. Defibrotide (Df) for the treatment of severe veno-occlusive disease (VOD) and multi-organ failure (MOF) post SCT: final results of a phase II, multi-center, randomized, dose-finding trial. Blood. 2006;108:438. [Google Scholar]
  57. Richardson P.G., Murakami C., Jin Z., et al. Multi-institutional use of defibrotide in 88 patients after stem cell transplantation with severe veno-occlusive disease and multisystem organ failure: response without significant toxicity in a high-risk population and factors predictive of outcome. Blood. 2002;100:4337–43. doi: 10.1182/blood-2002-04-1216. [DOI] [PubMed] [Google Scholar]
  58. Richardson P.G., Elias A.D., Krishnan A., et al. Treatment of severe veno-occlusive disease with defobrotide: compassionate use results in response without significant toxicity in a high-risk population. Blood. 1998;92:737–44. [PubMed] [Google Scholar]
  59. Ringden O., Remberger M., Lehmann S., et al. N-acetylcysteine for hepatic veno-occlusive disease after allogeneic stern cell transplantation. Bone Marrow Transplant. 2000;25:993–996. doi: 10.1038/sj.bmt.1702387. [DOI] [PubMed] [Google Scholar]
  60. Ringden O., Wennberg L., Ericzon B.G., et al. Altepiase for hepatic veno-occlusive disease after bane marrow transplantation. Lancet. 1992;340:546–547. doi: 10.1016/0140-6736(92)91741-P. [DOI] [PubMed] [Google Scholar]
  61. Rio B., Bauduer F., Arrago J.P., et al. N-terminal peptide of type III procollagen: a marker for the development of hepatic veno-occlusive disease after BMT and a basis for determining the timing of prophylactic heparin. Bone Marrow Transplant. 1993;11:471–2. [PubMed] [Google Scholar]
  62. Rio B., Andreu G., Nicod A., et al. Thrombocytopenia in venoocclusive disease after bane marrow transplantation or chemotherapy. Blood. 1986;67:1773–1776. [PubMed] [Google Scholar]
  63. Rodriguez-Inigo E., Tomas J.F., Gomez-Garcia de Soria V., et al. Hepatitis C and G virus infection and liver dysfunction after allogeneic bone marrow transplantation: results from a prospective study. Blood. 1997;90:1326–31. [PubMed] [Google Scholar]
  64. Rosenthai J., Sender L., Secola R., et al. Phase II trial of heparin prophylaxis for veno-occlusive disease of the liver in chidren undergoing bane marrow transplantation. Bone Marrow Transplant. 1996;18:185–191. [PubMed] [Google Scholar]
  65. Ruutu T., Eriksson B., Remes K., et al. Ursodeoxycholic acid for the prevention of hepatic complications in allogeneic stem cell transplantation. Blood. 2002;100:1977–83. doi: 10.1182/blood-2001-12-0159. [DOI] [PubMed] [Google Scholar]
  66. Sato Y., Asada Y., Hara S. e. aI. Hepatic stellate cells (Ito cells) in veno-occlusive disease of the liver after allogeneic bane marrow transplantation. Histopathology. 1999;34:66–70. doi: 10.1046/j.1365-2559.1999.00569.x. [DOI] [PubMed] [Google Scholar]
  67. Schlegel P.G., Haber H.P., Beck J., et al. Hepatic veno-occlusive disease in pediatric stem cell recipients: successful treatment with continuous infusion of prostaglandin E1 and low-dose heparin. Ann Hematol. 1998;76:37–41. doi: 10.1007/s002770050358. [DOI] [PubMed] [Google Scholar]
  68. Sebagh M., Oebette M., Samuel O., et al. Silent & qual presentation of veno-occlusive disease after liver transplantation as part of the process of cellular rejection with endothelial predilection. Hepatology. 1999;30:1144–1150. doi: 10.1002/hep.510300514. [DOI] [PubMed] [Google Scholar]
  69. Shulman H.M., Gooley T., Dudley M.D., et al. Utility of transvenous liver biopsies and wedged hepatic venous pressure measurements in sixty marrow transplant recipients. Transplantation. 1995;59:1015–22. doi: 10.1097/00007890-199504150-00017. [DOI] [PubMed] [Google Scholar]
  70. Shulman H.M., Fisher L.B., Schoch H.G., et al. Venoocclusive disease of the liver after marrow transplantation: Histological correlates of clinical signs ans symptoms. Hepatology. 1994;19:1171–80. doi: 10.1002/hep.1840190515. [DOI] [PubMed] [Google Scholar]
  71. Shulman H.M., Gown A.M., Nugent O.J. Hepatic veno-occlusive disease after bane marrow transplantation. Immunhistochemical identification of the material within occluded central venules. Am J Pathol. 1987;127:549–558. [PMC free article] [PubMed] [Google Scholar]
  72. Smith L.H., Dixon J.D., Stringham J.R., et al. Pivotal role of PAI-1 in a murine model of hepatic vein thrombosis. Blood. 2006;107:132–4. doi: 10.1182/blood-2005-07-2681. [DOI] [PMC free article] [PubMed] [Google Scholar]
  73. Srivastava A., Poonkuzhali B., Shaji R.V., et al. Glutathione S-tranferase M 1 polymorphism: a risk factor for hepatic veno-occlusive disease in bane marrow transplantation. Blood. 2004;104:1574–1577. doi: 10.1182/blood-2003-11-3778. [DOI] [PubMed] [Google Scholar]
  74. Strasser S.I., Myerson O., Spurgeon C.L., et al. Hepatitis C infection and bane marrow transplantation: a cohort study with 10-year follow-up. Hepatology. 1999;29:1893–1899. doi: 10.1002/hep.510290609. [DOI] [PubMed] [Google Scholar]
  75. Ulutin O.N. Antithrombotic effect and clinical potential of defibrotide. Semin Thromb Hemost. 1993;19(Suppl 1):186–91. [PubMed] [Google Scholar]
  76. Versluys B., Bhattacharaya R., Steward C., et al. Prophylaxis with defibrotide prevents veno-occlusive disease in stern Gell transplantation after gemtuzumab ozogamicin exposure. Blood. 2004;103:1986. doi: 10.1182/blood-2003-10-3612. [DOI] [PubMed] [Google Scholar]
  77. Wadleigh M., Richardson P.G., Zahrieh O., et al. Prior gemtuzumab ozogamicin exposure significantly increases the risk of veno-occlusive disease in patients who undergo myeloablative allogeneic stern Gell transplantation. Blood. 2003;102:1578–1582. doi: 10.1182/blood-2003-01-0255. [DOI] [PubMed] [Google Scholar]
  78. Wang X., Kanel G.C., OeLeve L.O. Support of sinusoidal endothelial Gell glutathione prevents hepatic veno-occlusive disease in the rat. Hepatology. 2000;31:428–434. doi: 10.1002/hep.510310224. [DOI] [PubMed] [Google Scholar]
  79. Williams L.M., Nelson S., Mason C.M., et al. Pulmonary veno-occlusive disease in an adult following bone marrow transplantatoin: Case reports and review of the literature. Chest. 1996;109:1388–91. doi: 10.1378/chest.109.5.1388. [DOI] [PubMed] [Google Scholar]
  80. Willmot F., Robertson G. Senecio disease, or cirrhosis of the liver due to senecio poisoning. Lancet. 1920;2:848–9. doi: 10.1016/S0140-6736(01)00020-4. [DOI] [Google Scholar]

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