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Hamostaseologie 2004; 24(04): 242-251
DOI: 10.1055/s-0037-1619641
Original Article
Schattauer GmbH

Erworbene Koagulopathien

Acquired coagulopathies
H. Riess
1   Medizinische Klinik mit Schwerpunkt Hämatologie und Onkologie, Campus Virchow-Klinikum, Universitätsklinikum Charité, Berlin
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Publication History

Publication Date:
03 February 2018 (online)

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Zusammenfassung

Erworbene Koagulopathien sind meist Folgen einer verursachenden Grundkrankheit (z. B. Leberinsuffizienz oder Sepsis). Die adäquate Behandlung dieser Grundkrankheit ist in der Regel von langfristig prognosebestimmender Bedeutung. Bei wenigen Koagulopathien, insbesondere bei den Immunkoagulopathien, findet sich aber keine erkennbare Grundkrankheit bei einem relevanten Anteil von Patienten. Die Optimierung der Hämostase mit dem Ziel fatale Blutungskomplikationen oder Thromboembolien zu verhindern, ist in beiden Fällen in der Lage, die Prognose zu verbessern. Meist gelingt dies durch individuell angepasste laborkontrollierte Therapien, die nur schwer einer Überprüfung im Rahmen von Studien zugänglich sind. In den vergangenen Jahren wurde erstmals in plazebokontollierten randomisierten Phase-III-Studien die Bedeutung der therapeutischen Hämostasemodulation zur Mortalitätsreduktion erfolgreich überprüft.

Summary

Acquired coagulopathies regularly result from underlying diseases such as liver failure or sepsis. Optimal treatment of these underlying diseases defines longterm prognosis. In a few coagulopathies, such as immunocoagulopathies, in a relevant portion of patients an underlying disease cannot be identified. Therapeutic modulation of haemostasis aiming at the prevention of fatal bleeding or thromboembolism is able to improve the prognosis of patients with acquired coagulopathies. Most often this is the result of individualized, lab-controlled treatments, which are difficult to be studied. Within the last years the role of therapeutic modulation of haemostasis to reduce mortality was successfully studied for the first time in the frame of randomized placebo-controlled phase III studies.

Schlüsselwörter

Erworbene Koagulopathie - Leberinsuffizienz - Niereninsuffizienz - DIC - erworbene Hemmkörper - Sepsis

Keywords

Acquired coagulopathy - liver failure - renal failure - DIC - acquired inhibitors - sepsis
 

  • Literatur

  • 1 Abraham E, Reinhart K, Opal S. et al. OPTI-MIST Trial Study Group. Efficacy and safety of tifacogin (recombinant tissue factor pathway inhibitor) in severe sepsis: a randomized controlled trial. JAMA 2003; 290: 238-47.
    CrossrefPubMedGoogle Scholar
  • 2 Abshire T, Kenet G. Recombinant factor VIIa: review of efficacy, dosing regimens and safety in patients with congenital and acquired factor VIII or IX inhibitors. J Thromb Haemost 2004; 2: 899-909.
    CrossrefPubMedGoogle Scholar
  • 3 Baca V, Montiel G, Meillon L. et al. Diagnosis of lupus anticoagulant in the lupus anticoagulant-hypoprothrombinemia syndrome: report of two cases and review of the literature. Am J Hematol 2002; 71: 200-7.
    CrossrefPubMedGoogle Scholar
  • 4 Briones M, Abshire T. Lupus anticoagulants in children. Curr Opin Hematol 2003; 10: 375-9.
    CrossrefPubMedGoogle Scholar
  • 5 Collins PW. Management of acquired haemophilia A – more questions than answers. Blood Coagul Fibrinolysis 2003; 14 (Suppl. 01) S23-7.
    CrossrefPubMedGoogle Scholar
  • 6 Delgado J, Jimenez-Yuste V, Hernandez-Navarro F. et al. Acquired haemophilia: review and meta-analysis focused on therapy and prognostic factors. Br J Haematol 2003; 121: 21-35.
    CrossrefPubMedGoogle Scholar
  • 7 Derksen RH, Khamashta MA, Branch DW. Management of the obstetric antiphospholipid syndrome. Arthritis Rheum 2004; 50: 1028-39.
    CrossrefPubMedGoogle Scholar
  • 8 Dhainaut JF, Laterre PF, Janes JM. et al. Recombinant Human Activated Protein C Worldwide Evaluation in Sepsis (PROWESS) Study Group. Drotrecogin alfa (activated) in the treatment of severe sepsis patients with multiple-organ dysfunction: data from the PRO-WESS trial. Intensive Care Med 2003; 29: 894-903.
    CrossrefPubMedGoogle Scholar
  • 9 Dhainaut JF, Yan SB, Margolis BD. et al. PRO-WESS Sepsis Study Group. Drotrecogin alfa (activated) (recombinant human activated protein C) reduces host coagulopathy response in patients with severe sepsis. Thromb Haemost 2003; 90: 642-53.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 10 Eby C, Blinder M. Hemostatic complications associated with paraproteinemias. Curr Hematol Rep 2003; 2: 388-94.
    PubMedGoogle Scholar
  • 11 Federici AB, Budde U, Rand JH. Acquired von Willebrand syndrome 2004: International Registry – diagnosis and management from online to bedside. Hämostaseologie 2004; 24: 50-5.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 12 Freeman BD, Buchman TG. Coagulation inhibitors in the treatment of sepsis. Expert Opin Investig Drugs 2002; 11: 69-74.
    CrossrefPubMedGoogle Scholar
  • 13 Gezer S. Antiphospholipid syndrome. Dis Mon 2003; 49: 696-741.
    CrossrefPubMedGoogle Scholar
  • 14 Kang Y. Coagulopathies in hepatic disease. Liver Transpl 2000; 6 (Suppl. 01) S72-5.
    CrossrefPubMedGoogle Scholar
  • 15 Kerr R. New insights into haemostasis in liver failure. Blood Coagul Fibrinolysis 2003; 14 (Suppl. 01) S43-5.
    CrossrefPubMedGoogle Scholar
  • 16 Key NS, Ely EW. Coagulation inhibition for sepsis. Curr Opin Hematol 2002; 9: 416-21.
    CrossrefPubMedGoogle Scholar
  • 17 Levi M, de Jonge E, van der Poll T. New treatment strategies for disseminated intravascular coagulation based on current understanding of the pathophysiology. Ann Med 2004; 36: 41-9.
    PubMedGoogle Scholar
  • 18 Mousa SA. Antithrombotics in thrombosis and cancer. Expert Rev Cardiovasc Ther 2003; 1: 283-91.
    CrossrefPubMedGoogle Scholar
  • 19 Opatrny Jr. K. Hemostasis disorders in chronic renal failure. Kidney Int Suppl 1997; 62: S87-9.
    PubMedGoogle Scholar
  • 20 Ozturk MA, Haznedaroglu IC, Turgut M. et al. Current debates in antiphospholipid syndrome: the acquired antibody-mediated thrombophilia. Clin Appl Thromb Hemost 2004; 10: 89-126.
    CrossrefPubMedGoogle Scholar
  • 21 Passam F, Krilis S. Laboratory tests for the antiphospholipid syndrome: current concepts. Pathology 2004; 36: 129-38.
    CrossrefPubMedGoogle Scholar
  • 22 Polderman KH, Girbes AR. Drug intervention trials in sepsis: divergent results. Lancet 2004; 363 (9422): 1721-3.
    CrossrefPubMedGoogle Scholar
  • 23 Riewald M, Riess H. Treatment options for clinically recognized disseminated intravascular coagulation. Semin Thromb Hemost 1998; 24: 53-9.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 24 Shearer MJ. Vitamin K. Lancet 1995; 345 (8944): 229-34.
    CrossrefPubMedGoogle Scholar
  • 25 Schetz MR. Coagulation disorders in acute renal failure. Kidney Int Suppl 1998; 66: S96-101.
    PubMedGoogle Scholar
  • 26 Slofstra SH, Spek CA, ten Cate H. Disseminated intravascular coagulation. Hematol J 2003; 4: 295-302.
    CrossrefPubMedGoogle Scholar
  • 27 Stasi R, Brunetti M, Stipa E. et al. Selective B-cell depletion with rituximab for the treatment of patients with acquired hemophilia. Blood 2004; 103: 4424-8.
    CrossrefPubMedGoogle Scholar
  • 28 Triplett DA. Lupus anticoagulants: diagnosis and management. Curr Hematol Rep 2003; 2: 271-2.
    PubMedGoogle Scholar
  • 29 Warren BL, Eid A, Singer P. et al. KyberSept Trial Study Group. Caring for the critically ill patient. High-dose antithrombin III in severe sepsis: a randomized controlled trial. JAMA 2001; 286: 1869-78.
    CrossrefPubMedGoogle Scholar
  • 30 White B, Perry D. Acquired antithrombin deficiency in sepsis. Br J Haematol 2001; 112: 26-31.
    CrossrefPubMedGoogle Scholar
  • 31 Zacharski LR. Malignancy as a solid-phase coagulopathy: implications for the etiology, pathogenesis, and treatment of cancer. Semin Thromb Hemost 2003; 29: 239-46.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 32 Zangari M, Saghafifar F, Mehta P. et al. The blood coagulation mechanism in multiple myeloma. Semin Thromb Hemost 2003; 29: 275-82.
    Article in Thieme ConnectPubMedGoogle Scholar
  • 33 Zakarija A, Kwaan HC. Bleeding and thrombosis in the cancer patient. Expert Rev Cardiovasc Ther 2003; 1: 271-81.
    CrossrefPubMedGoogle Scholar
  • 34 Zakarija A, Green D. Acquired hemophilia: diagnosis and management. Curr Hematol Rep 2002; 1: 27-33.
    PubMedGoogle Scholar