Labordiagnostik von Lupushemmstoffen

Zusammenfassung

Das Antiphospholipidsyndrom wird definiert als eine Erkrankung mit rezidivierenden Thrombosen und/oder ungeklärten Spontanaborten sowie dem gleichzeitigen Nachweis von Antiphospholipid-Antikörpern. Eine Untergruppe dieser Antintikörper zeichnet sich dadurch aus, dass sie zu einer Verlängerung der Gerinnungszeit von phospholipidabhängigen Gerinnungstests führen. Diese unspezifischen Inhibitoren werden, auf Grund ihrer Erstbeschreibung von Conley und Hartmann in einem Kollektiv von Patienten mit systemischen Lupus erythematodes, als Lupushemmstoffe bezeichnet.

Lupushemmstoffe sind erworbene Autoantikörper, die gegen Phospholipid-bindende Proteine (z. B. β2-Glykoprotein I, Prothrombin) gerichtet sind. Durch diese Bindung werden bivalente Komplexe auf gerinnungsaktiven Phospholipiden gebildet, die zu einer Verlangsamung des Gerinnungsablaufes führen. In vivo können diese Komplexe zu einer Zellaktivierung und in der Folge zu Thrombosen führen.

Die Labordiagnose von Lupushemmstoffen wird durch eine Testkombination gestellt: phospholipidabhängige Gerinnungstests, Plasmatauschversuch und Nachweis der Phospholipidabhängigkeit des Hemmstoffs.

Summary

The antiphospholipid syndrome is defined as the occurrence of one or more episodes of vascular thrombosis and/or miscarriage together with antiphospholipid antibodies. Some of these antibodies can be detected via coagulation assays by prolonging phospholipids-dependent assays. These unspecific inhibitors are termed lupus anticoagulant, due to their first description by Conley and Hartmann in patients with disseminated lupus erythematosus.

Lupus anticoagulants are now defined as acquired autoantibodies directed against a phospholipid-binding protein such as β2-glycoprotein I or prothrombin. Because of this binding, lupus anticoagulants form bivalent complexes which slow down coagulation reactions in vitro by forming stable complexes on coagulation active phospholipids. In vivo, these complexes may result in cellular activation and cause thrombosis.

Laboratory diagnosis for the lupus anticoagulant should be performed by a combination of tests, including phospholipid-dependent clotting assays, plasma mixing studies, and demonstration of the phospholipid-dependency of the inhibitory activity.

Schlüsselwörter

Lupushemmstoff - Antiphospholipidsyndrom

Keywords

Lupus anticoagulant - antiphospholipid syndrome

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