Anticoagulation therapy in haemophilia

Summary

Patients with haemophilia (PWH) are relatively protected from cardiovascular death. Recent insights have shown that this is not due to less formation of atherosclerosis than in non-haemophilic men, therefore protection from the final occlusive thrombus will be the major determinant. Prevalence and incidence rates of cardiovascular disease (especially non-fatal events) are scarce, although ongoing studies are addressing this issue.

Meanwhile, because the haemophilia population is aging, we are increasingly confronted with cardiovascular events. The main cardio vascular risk factors that should be part of regular screening programs are hypertension, overweight, lipometabolic disorders and smoking. Anticoagulation therapy in haemophilia is feasible, provided that individual tailored coagulation therapy and close monitoring is provided.

Here, we present our view on anticoagulation management in PWH. There is an absolute need for risk assessment tools and prospective validation of suggested anticoagulation management strategies in PWH. Until then, we are managing the unknown.

Zusammenfassung

Patienten mit Hämophilie sind relativ geschützt vor kardiovaskulär bedingtem Tod. Nach neuen Erkenntnissen ist dies nicht darauf zurückzuführen, dass die Arteriosklerose geringer ausgeprägt ist als bei Männern ohne Hämophilie, daher wird der Schutz vor dem letztlich okklusiven Thrombus der ausschlaggebende Faktor sein. Daten zur Prävalenz und Inzidenz kardiovaskulärer Erkrankungen (besonders für nicht tödliche Ereignisse) liegen kaum vor, jedoch werden zurzeit Studien dazu durchgeführt.

Inzwischen sehen wir uns angesichts der alternden, von Hämophilie betroffenen Population zunehmend mit kardiovaskulären Ereignissen konfrontiert. Die wichtigsten kardiovaskulären Risikofaktoren eines regelmäßigen Screening Programms sollten Hypertonie, Übergewicht, metabolisches Syndrom und Rauchen sein. Eine antikoagulatorische Therapie der Hämophilie ist möglich, wenn sie individuell angepasst wird und unter engmaschiger Kontrolle erfolgt.

Hier legen wir unsere Sicht der antikoagulatorischen Behandlung von Hämophilie-Patienten dar. Es besteht ein Bedarf an Methoden zur Risikobeurteilung und zur prospektiven Validierung der für Hämophilie-Patienten vorgeschlagenen Behandlungsstrategien zur Antikoagulation. Bis dahin bewegen wir uns auf unbekanntem Terrain.

Keywords

Haemophilia - anticoagulation - aspirin - coumarin - cardiovascular disease - atrial fibrillation

Schlüsselwörter

Hämophilie - Antikoagulation - Azetlysalizylsäure - Cumarin - kardiovaskuläre Erkrankung - Vorhofflimmern

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