Therapie bei erworbenen Inhibitoren gegen plasmatische Gerinnungsfaktoren

Zusammenfassung

Spezifische, neutralisierende Inhibitoren und unspezifische Inhibitoren, die durch Komplexbildung mit einem Gerinnungsfaktor und dessen rascher Elimination ebenfalls zu Faktormangel führen, können schwere Blutungen verursachen. Diagnostisch müssen sie unterschieden werden von Antiphospholipid-Antikörpern, die nicht zu einer hämorrhagischen Diathese führen. Aufgrund der heutigen Kenntnisse und eigener Beobachtungen an solchen Patienten werden die aktuellen Behandlungsstrategien erläutert: 1. Allgemeine Maßnahmen (Absetzen von Medikamenten, welche die Gerinnung zusätzlich beeinträchtigen, Unterlassen von nicht vital notwendigen diagnostischen oder therapeutischen Eingriffen), 2. Spezifische Blutstillung mit Gerinnungspräparaten, soweit diese nötig ist; bei den häufigsten Inhibitoren gegen Faktor VIII vorzugsweise mit rekombinantem aktiviertem Faktor VII (NovoSeven^®), da dieser den Inhibitor nicht stimuliert. 3. Maßnahmen zur Elimination des Inhibitors: Immunsuppressiva, eventuell kombiniert mit Apherese und Immunadsorption, in Ergänzung zur Behandlung der zugrundeliegenden Krankheit, falls eine solche möglich ist.

Summary

Specific neutralizing inhibitors and unspecific inhibitors, which eliminate coagulation factors by complex formation, lead to factor deficiencies with the risk of severe bleeding. They have to be distinguished from antiphospholipid antibodies with an in vitro inhibiting effect only. Treatment strategies are explained basing upon current knowledge and our proper observations in these patients: 1. General measures: To avoid medication which impairs blood coagulation and to avoid non-vital invasive diagnostic and therapeutic interventions. 2. Specific haemostasis with coagulation concentrates, if necessary. In case of the most frequent inhibitors against factor VIII preferably recombinant activated factor VII (Novo Seven^®), since NovoSeven does not stimulate inhibitor formation. 3. Measures to eliminate the inhibitor: Immunosuppressives, with or without apheresis and immunoadsorption, if possible in addition to the treatment of the underlying disorder.

Schlüsselwörter

Blutgerinnung - Inhibitoren - Faktor-VIII-Inhibitoren - Von-Willebrand-Syndrom

Keywords

Blood coagulation - inhibitors - factor VIII inhibitors - von Willebrand's disease.

• Literatur

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