علاج انحلال الدم الليلي الانتيابي مع الانسداد الوريدي الخثاري

References

Venous thromboembolism or previous venous thromboembolism or increased risk (see chapter 4.1.2)

In the event of a thromboembolic event as the first manifestation of PNH, immediate interruption of intravascular haemolysis (IVH) is necessary.

In the event of a thromboembolic event as the first manifestation of PNH, immediate interruption of intravascular haemolysis (IVH) is necessary. According to current data, this is most reliably achieved by administering an effective terminal complement inhibitor (e.g. ravulizumab), as thrombin can directly activate C3 and C5 and plasmin also activates C5, making rapid C5 blockade useful.

Long-term or lifelong anticoagulation after a thrombosis is recommended.

Both coumarins and heparins can be used therapeutically and prophylactically.

Published data on the use of DOACs are only available for very small groups of patients, but their use, especially with simultaneous administration of complement inhibitors, seems to lead to comparable results based on experience in centers.

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