Wolfgang Miesbach: Hereditary Hemorrhagic Telangiectasia An Overlooked Bleeding Disorder
Wolfgang Miesbach, Head of Hemostasis/Hemophilia Center at Medical Clinic 2 at University Hospital Frankfurt, shared a post on LinkedIn about a recent article by Hanny Al-Samkari, published in The Lancet Haematology, adding:
”Twice as common as haemophilia, no licensed drug anywhere, six new agents in development.
Hanny Al-Samkari new review in The Lancet Haematology (online 11 October) is a reminder of how neglected HHT has been.
It is the second most common heritable bleeding disorder and carries the highest morbidity of any inherited bleeding disorder in women.
The burden:
- Three in four patients develop progressive mucosal bleeding
- Most become iron deficient, and their IV iron and transfusion needs exceed those of other bleeding disorders
- One in 30 has an intracranial haemorrhage
- Up to one in six develops venous or arterial thromboembolism, so anticoagulation becomes a daily dilemma
Why we miss it:
The coagulation screen is normal.
The diagnosis is in the history and on the lips, tongue and fingertips.
Once disease-specific drugs arrive, an undiagnosed patient becomes an untreated one.”
Title: Emerging novel therapies for the treatment of hereditary haemorrhagic telangiectasia
Authors: Hanny Al-Samkari
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