Recombinant ADAMTS13 should be the standard of care for patients with congenital TTP. FFP is an inferior product both in terms of efficacy and safety.
A paper in the journal Blood describes the experience from Japan in 14 patients.
lnkd.in/eXg345SD

Avui 17 d’abril dia mundial de la Hemofília: Des de l’Hospital de Sant Pau ens sumem a l’accés per a tothom amb coagulopaties congènites! #WeBleedToo #whd2025 @hemofilia_cat @HemostasiaStPau @hospitalsantpau.bsky.social

Check out this super cool video on a recently published @jthjournal.bsky.social journal article: www.youtube.com/watch?v=Zx4z.... Read the article here: bit.ly/4g4gYqX

Only if combined with progestogen. Transdermal oestrogen alone (the bottom group) had no increase in risk of venous thrombosis, confirming the findings of other studies.

It is great to see the organisations and journals I closely work with joining BlueSky.
@isth-official.bsky.social
@eahad.bsky.social
@rpth.bsky.social
@jthjournal.bsky.social
If you are involved in thrombosis and hemostasis or hemophilia, consider following them.

Acquired hemophilia is a severe bleeding disorder that is difficult to treat. This review provides an algorithmic approach to treatment that includes emicizumab, limits hemostatic product use, and minimizes immunosuppression toxicity.

www.jthjournal.org/article/S153...

#MedSky #HemeSky

Paper in the journal BloodAdvances discusses liver disease in the context of gene therapy (GT) for #hemophilia. Who should not have GT and post-GT, how should ALT elevations be managed?https://tinyurl.com/bdeav8kj @bloodadvances.bsky.social

The World Federation of #Hemophilia 2023 Annual Global Survey was published today. These are the best international data on inherited bleeding disorders. Europe and the Americas have 28% of the world population and use 79% of the world's FVIII concentrate. tinyurl.com/4muevre6

The last two years have seen significant advances in our understanding of fibrinogen and fibrin structure and function. In this review, the authors discuss recent progress and suggest novel research avenues for future studies.

www.jthjournal.org/article/S153...

#MedSky #Fibrinogen #HemeSky