Hamostaseologie 1995; 15(02): 100-104
DOI: 10.1055/s-0038-1655294
Labordiagnostik/Laboratory Diagnostics
Schattauer GmbH

Lupus-Antikoagulans: Klinik, Pathophysiologie, Diagnostik und Therapie

B. Pötzsch
1   Abteilung Hämostaseologie und Transfusionsmedizin (Direktor: Prof. Dr. G. MüllerBerghaus), Kerckhoff-Klinik, Max-Planck- Institut für physiologische und klinische Forschung, Bad Nauheim.
,
K. Madlener
1   Abteilung Hämostaseologie und Transfusionsmedizin (Direktor: Prof. Dr. G. MüllerBerghaus), Kerckhoff-Klinik, Max-Planck- Institut für physiologische und klinische Forschung, Bad Nauheim.
› Author Affiliations
Further Information

Publication History

Publication Date:
26 June 2018 (online)

Zusammenfassung

Ein Lupus-Antikoagulans (LA) ist durch das Auftreten von Autoantikörpern, die gegen gerinnungsaktive Phospholipide gerichtet sind, charakterisiert. Typischerweise findet sich bei betroffenen Patienten eine Verlängerung Phospholipidabhängiger Gerinnungsteste, während klinisch eine Thrombose- und Abortneigung überwiegt. Pathophysiologisch konnte als eine Ursache der Thromboseneigung eine Inhibierung der antikoagulatorischen Aktivität von aktiviertem Protein C nachgewiesen werden. Andere Ursachen sind bisher noch unklar. Die sichere Diagnostik des LA erfordert die Kombination zweier voneinander unabhängiger funktioneller Teste oder die Kombination eines funktioneilen mit einem immunologischen Testverfahren. Therapeutisches Ziel bei LA-Patienten ist die Prophylaxe thrombotischer Komplikationen durch eine adäquate Antikoagulation.

 
  • LITERATUR

  • 1 Feinstein DL. Lupus anticoagulant, anticardiolipin antibodies, fetal loss, and systemic lupus erythematosus. Blood 1992; 80: 859-62.
  • 2 Harris EN. Antiphospholipid antibodies. Br J Haematol 1990; 74: 1-9.
  • 3 Pengo V, Thiagarajan P, Shapiro SS, Heine MJ. Immunological specificity and mechanism of action of IgG lupus anticoagulants. Blood 1987; 70: 69-76.
  • 4 Galli M, Bevers EM. Inhibition of phospholipid-dependent coagulation reactions by »Antiphospholipid Antibodies«: Possible modes of action. Lupus 1994; 03: 223-8.
  • 5 Mueller JF, Ratnoff O, Heinle RW. Observations on the characteristics of an unusual circulating anticoagulant. Lab Clin Med 1951; 38: 254-61.
  • 6 Conley CL, Hartmann RC. Hemorrhagic disorder caused by circulating anticoagulant in patients with disseminated lupus erythematosus. J Clin Invest 1952; 150: 621-2.
  • 7 Bowie WEJ, Thompson JH, Pascuzzi CA, Owen GA. Thrombosis in systemic lupus erythematosus despite circulating anticoagulants. J Clin Invest 1963; 62: 416-30.
  • 8 Lo SCL, Salem HH, Howard MA. et al. Studies of natural anticoagulant proteins and anticardiolipin antibodies in patients with the lupus anticoagulant. Br J Haematol 1990; 76: 380-6.
  • 9 Ninomiya C, Taniguchi O, Kato T. et al. Distribution and clinical significance of lupus anticoagulant and anticardiolipin antibody in 349 patients with systemic lupus erythematosus. Int Med 1992; 31: 194-9.
  • 10 Jouhikainen T, Julkunen H, Vaarala O. et al. Antiphospholipid antibodies and thrombosis in systemic lupus erythematosus: comparison of three lupus anticoagulant assays and anticardiolipin ELISA in 188 patients. Blood Coagulation and Fibrinolysis 1992; 03: 407-14.
  • 11 Kornberg A, Silber L, Yona R, Kaufman S. Clinical manifestations and laboratory findings in patients with lupus anticoagulants. Eur J Haematol 1989; 42: 90-5.
  • 12 McGee GS, Pearce WH, Sharma L. et al. Antiphospholipid antibodies and arterial thrombosis. Arch Surg 1992; 127: 342-6.
  • 13 Kleiner RC, Najarian LV, Schatten S. et al. Vaso-occlusive retinopathy associated with anti-phospholipid antibodies (lupus anticoagulant retinopathy). Ophthalmology 1989; 96: 896-904.
  • 14 Toyoshima K, Makino T, Ozawa N. et al. Effect of anticardiolipin antibody in patients with recurrent fetal loss on thrombomodulin-dependent protein C activation. J Clin Lab Anal 1993; 07: 57-9.
  • 15 Asherson RA, Mayou SC, Merry P. et al. The spectrum of livedo reticularis and anticardiolipin antibodies. Br J Dermatol 1989; 120: 215-21.
  • 16 Fleck RA, Rapaport SI, Mohan LVRao. Anti-Prothrombin antibodies and the lupus anticoagulant. Blood 1988; 72: 512-9.
  • 17 Nilsson TK, Löfvenberg E. Decreased fibrinolytic capacity and increased von Willebrand factor levels as indicators of endothelial cell dysfunction in patients with lu-pus anticoagulant. Clin Rheumatol 1989; 08: 58-63.
  • 18 Tsakiris DA, Marbet GA, Makris PE. et al. Impaired fibrinolysis as an essential contribution to thrombosis in patients with lupus anticoagulant. Thromb Haemost 1989; 61: 175-7.
  • 19 Francis RB, McGehee WG, Feinstein DI. Endothelial-dependent fibrinolysis in subjects with the lupus anticoagulant and thrombosis. Thromb Haemost 1988; 59: 412-4.
  • 20 Watson KV, Schorer AE. Lupus anticoagulant inhibition of in vitro prostacyclin release is associated with a thrombosis-prone subset of patients. Am J Med 1992; 90: 47-53.
  • 21 Dudley DJ, Mitchell MD, Branch DW. Pathophysiology of antiphospholipid antibodies: Absence of prostaglandin-mediated effects on cultured endothelium. Am J Obstet Gynecol 1990; 162: 953-9.
  • 22 Coade SB, van Haaren E, Loizou S. et al. Endothelial prostacyclin release in systemic lupus erythematosus. Thromb Haemost 1989; 61: 97-100.
  • 23 Schorer AE, Wickham WR, Watson KV. Lupus anticoagulant induces a selective defect in thrombin-mediated endothelial prostacyclin release and platelet aggregation. Br J Haematol 1989; 71: 399-407.
  • 24 Lellouche F, Martinuzzo M, Said P. et al. Imbalance of thromboxane/prostacyclin bio-synthesis in patients with lupus anticoagulant. Blood 1991; 78: 2894-9.
  • 25 Vismara A, Meroni PL, Tincani A. et al. Relationship between anti-cardiolipin and anti-endothelial cell antibodies in systemic lupus erythematosus. Clin Exp Immunol 1988; 74: 247-53.
  • 26 Tsakiris DA, Settas L, Makris PE, Marbet GA. Lupus anticoagulant – Antiphospholipid antibodies and thrombophilia. Relation to protein C – protein S – thrombomodulin. J Rheumatol 1990; 17: 785-9.
  • 27 Harrison RL, Alperin JB. Concurrent protein C deficiency and lupus anticoagulants. Am J Hematol 1992; 40: 33-7.
  • 28 Freyssinet JM, Cazenave JP. Lupus-like anticoagulants, modulation of the protein C pathway and thrombosis. Thromb Haemost 1987; 58: 679-81.
  • 29 Ruiz-Arguelles GJ, Ruiz-Arguelles A, Deleze M, Alarcön-Segovia D. Acquired protein C deficiency in a patient with primary antiphospholipid syndrome. Relationship to reactivity of anticardiolipin antibody with thrombomodulin. J Rheum 1989; 16: 381-3.
  • 30 Borrell M, Sala N, de Castellarnau C. et al. Immunoglobulin fractions isolated from patients with antiphospholipid antibodies prevent the inactivation of factor Va by activated protein C on human endothelial cells. Thromb Haemost 1992; 68: 268-72.
  • 31 Pötzsch B, Kawamura H, Preissner KT. et al. Thrombophilia in patients with lupus anticoagulant correlates with impaired anticoagulant activity of activated protein C but not with decreased activation of protein C. Blood 1992; 80: 267a (Abstract)
  • 32 Rosner E, Pauzner R, Lusky A. et al. Detection and quantitative evaluation of lupus circulating anticoagulant activity. Thromb Haemost 1987; 57: 144-7.
  • 33 Rauch J, Tannenbaum M, Janoff AS. Distinguishing plasma lupus anticoagulants from anti-factor antibodies using hexagonal (II) phase phospholipids. Thromb Haemost 1989; 62: 892-6.
  • 34 Landy HJ, Kessler C, Kelly WK, Weingold AB. Obstetric performance in patients with the lupus anticoagulant and/or anticardiolipin antibodies. Am J of Perinatol 1992; 09: 146-51.