Emeritus Professor of Haemostasis and Thrombosis in Sheffield, UK. I tweet about thrombosis, hemophilia, COVID-19, medicine, NHS & AI. Opinions are my own.https://bsky.app/profile/profmakris.bsky.socialhttps://bsky.app/profile/profmakris.bsky.social/post/3lpa46o52kk2lRecombinant 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/eXg345SD15 May 2025 18:07 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3lpa46o52kk2lhttps://bsky.app/profile/profmakris.bsky.social/post/3lp4vogmuxs23For anybody interested in birds, this is a great book. The author tried to learn more about birds by building a shed/hide close to his home in the south of England. Very well researched and informative. I learnt a lot about the birds we encounter in the UK.14 May 2025 11:32 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3lp4vogmuxs23https://bsky.app/profile/profmakris.bsky.social/post/3lolmpz7cos2dIf you are interested in birds, the Greek island of Lesbos is a great place to visit this time of the year, as it coincides with the spring migration. We saw 170 species of bird in one week thanks to the skills of "Mr Lesbos Birding" Howard Vaughan, who also took the bird pictures below.07 May 2025 14:37 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3lolmpz7cos2dhttps://bsky.app/profile/profmakris.bsky.social/post/3lofowcj5vs2jMy commentary on PITT (PF4-associated immune thrombocytopenia and thrombosis) has just been published in the British Journal of Haematology. This group of disorders is more than HIT and VITT and we should be looking for it more widely. https://onlinelibrary.wiley.com/doi/10.1111/bjh.2012505 May 2025 06:00 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3lofowcj5vs2jhttps://bsky.app/profile/profmakris.bsky.social/post/3lncy5t2by22yOur editorial on the Paradox of Choice in Hemophilia has just been published in the journal Research and Practice in Thrombosis and Haemostasis. Like all papers in this journal, it is freely available. tinyurl.com/4kw9hcth @rpth.bsky.social #hemophilia #haemophilia21 Apr 2025 10:42 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3lncy5t2by22yhttps://bsky.app/profile/profmakris.bsky.social/post/3ln3pdxat2k2qThank you. In addition, there were no cases of malignancy following gene therapy, but the number of patients and years of follow-up is small. [contains quote post or other embedded content]18 Apr 2025 13:16 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3ln3pdxat2k2qhttps://bsky.app/profile/profmakris.bsky.social/post/3ln3awxijcc2qA paper in @NEJM describes 14 patients with #hemophilia B who had the Pfizer gene therapy. Their achieved FIX levels remained stable over a median of 5.5 years. Confirmation that durability following hemophilia B gene therapy is very different to that in hemophilia A. tinyurl.com/2b2zzc3718 Apr 2025 08:58 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3ln3awxijcc2qhttps://bsky.app/profile/profmakris.bsky.social/post/3ln3absx7xc2qAn article in the current issue of @nejm.org discusses the impact of the US foreign assistance cuts on global health. Some of the impacts are not readily obvious. The authors advocate reforming the foreign assistance program rather than dismantling it.18 Apr 2025 08:46 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3ln3absx7xc2qhttps://bsky.app/profile/profmakris.bsky.social/post/3ln35yhdvo22qThe BIC meeting in Italy is always a great meeting, showcasing the latest science and with plenty of time for discussion. Remember the deadline for abstract submission is the 30th April 2025. #hemophilia #VWD #TMA #TTP18 Apr 2025 08:05 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3ln35yhdvo22qhttps://bsky.app/profile/profmakris.bsky.social/post/3lmyn6h4tas2qToday is #WorldHemophiliaDay and this year's theme is that women and girls bleed too. Despite the development of brilliant treatments to treat severe #hemophilia primarily in men, the diagnosis and treatment of women have not received as much attention.17 Apr 2025 07:59 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3lmyn6h4tas2qhttps://bsky.app/profile/profmakris.bsky.social/post/3lmjjrszap22wThis is the press release about the new #hemophilia B gene therapy that has been approved in China. So there are now 3 approved gene therapies for hemophilia B, 2 in US/Europe approved by the FDA/EMA and 1 in China approved by the Chinese regulator. tinyurl.com/2jc74nb9 https://tinyurl.com/2jc74nb911 Apr 2025 07:49 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3lmjjrszap22whttps://bsky.app/profile/profmakris.bsky.social/post/3lmicyxnzkc25Thank you for the copies of the paper on the UK Infected Blood Inquiry that was published in @BloodJournal. It is interesting to see the section on lessons learned. Despite the @bloodinquiry, the UK continues to use cryo and FFP without viral inactivation. #hemophilia10 Apr 2025 20:15 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3lmicyxnzkc25https://bsky.app/profile/profmakris.bsky.social/post/3lmhwyslo4s25Article on Generative AI in medicine published today in @nejm.org. It discusses the potential uses and risks. One issue you can be sure of, is that AI will be embedded in the everyday practice of medicine in the very near future, even more than today. tinyurl.com/4sbjpjvs10 Apr 2025 16:40 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3lmhwyslo4s25https://bsky.app/profile/profmakris.bsky.social/post/3lmddaopf322zIf you are a scientist who is not using AI yet, this article from today's @nature.com journal will be of great help. Whether we like it or not, AI will be everywhere very soon, just like the internet.08 Apr 2025 20:36 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3lmddaopf322zhttps://bsky.app/profile/profmakris.bsky.social/post/3lm5ech6ils2cEverybody has an opinion about racism, but as the author of this book explains, the plural of anecdote is not data. This is a serious book looking at the scientific evidence around racism. It will not come as a surprise to read that racism is still in everything.06 Apr 2025 11:39 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3lm5ech6ils2chttps://bsky.app/profile/profmakris.bsky.social/post/3llyj2b3bp22kVaccination skeptics should have a look at this figure. It shows the impact of the introduction of RSV vaccination for patients aged 75-80 on hospital admissions in the UK. tinyurl.com/5d48u7mm04 Apr 2025 13:20 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3llyj2b3bp22khttps://bsky.app/profile/profmakris.bsky.social/post/3llws52s2bc2uImportant paper: In patients with cancer and venous thrombosis, extended anticoagulation with low dose Apixaban is as good as full dose Apixaban in preventing thrombosis recurrence and is associated with less bleeding. tinyurl.com/yyfuan3503 Apr 2025 20:57 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3llws52s2bc2uhttps://bsky.app/profile/profmakris.bsky.social/post/3llwqptpyd22uProfessor Christine Lee reflects on some of the conclusions of the UK Infected Blood Inquiry. The manuscript has just been published by the journal Haemophilia. @haemophilia_jnl #hemophilia. The PDF of the manuscript is freely available at: tinyurl.com/mvh2mk2203 Apr 2025 20:32 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3llwqptpyd22uhttps://bsky.app/profile/profmakris.bsky.social/post/3llwq4ygmtk2uOur paper on the severity of #hemophilia has just been published in the journal Hemasphere. Baseline clotting factors do not always accurately reflect the severity of the disease. tinyurl.com/3hn9hhrn https://tinyurl.com/3hn9hhrn03 Apr 2025 20:22 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3llwq4ygmtk2uhttps://bsky.app/profile/profmakris.bsky.social/post/3llwmqkkljc2uOur paper on inhibitor trends is now published in the journal Haemophilia. The number of inhibitors in previously untreated patients has markedly reduced, but it is possible that this is due to delayed exposure to FVIII. tinyurl.com/2mr3t5e303 Apr 2025 19:21 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3llwmqkkljc2uhttps://bsky.app/profile/profmakris.bsky.social/post/3llwmancfy22uThe German authorities have today concluded their negotiations with CSL Behring and agreed the price they will pay for the #hemophilia B gene therapy (Hemgenix). Does anybody know how much they will pay? tinyurl.com/mpnc9kk7 https://tinyurl.com/mpnc9kk703 Apr 2025 19:12 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3llwmancfy22uhttps://bsky.app/profile/profmakris.bsky.social/post/3llpajo62dc2sComprehensive review of gene therapy in #hemophilia accepted for publication by Molecular Therapy. The pre-proof is on line at tinyurl.com/3sb4nuex31 Mar 2025 20:54 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3llpajo62dc2shttps://bsky.app/profile/profmakris.bsky.social/post/3llp5dta2xk2nPaying reviewers to assess papers submitted to scientific journals results in more rapid reviews, but the quality does not change. News article in @nature.com. tinyurl.com/bdfxf9xy https://tinyurl.com/bdfxf9xy31 Mar 2025 19:57 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3llp5dta2xk2nhttps://bsky.app/profile/profmakris.bsky.social/post/3llmconmrv22aIncredible: https://dropbox.com/scl/fi/qy2ja10p9pnolo2wussb3/Hemophilia-Treatment-Evolution_-Progress-and-the-Paradox-of-Choice.wav?rlkey=vgi323zzgk17vhcp9z18fo0yn&dl=0. This podcast was produced 100% by AI. It is entirely based on the editorial by myself and Brian O'Mahony published in @rpth.bsky.social. It is 21 min and remarkably accurate. Thank you, Eugene Fan, for showing me what is possible.30 Mar 2025 16:54 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3llmconmrv22ahttps://bsky.app/profile/profmakris.bsky.social/post/3lllf4liupk2bThis is an amazingly prophetic book written by two Harvard professors who teach political science. Although published in 2018, it clearly describes what is happening in the world and the US in particular today. "History does not repeat itself; it rhymes."30 Mar 2025 08:05 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3lllf4liupk2bhttps://bsky.app/profile/profmakris.bsky.social/post/3llhwuc7twc2qAs expected the FDA has today announced that it has approved Fitusiran for individuals aged 12+ with #hemophilia A or B with or without inhibitors. Commercial name is Qfitlia - they must have tried hard to find a difficult to pronounce name. tinyurl.com/mr4xmawt https://tinyurl.com/mr4xmawt28 Mar 2025 23:12 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3llhwuc7twc2qhttps://bsky.app/profile/profmakris.bsky.social/post/3llf7t6vthk2dTomorrow, March 28th, is the big day for Fitusiran as the FDA is expected to give their decision. Will they approve it? Probably. Will it have a black box warning? We do not know, but since they did not use them for Marstacimab and Concizumab, they will probably not. #hemophilia27 Mar 2025 21:14 +0000at://did:plc:4ajh6cilygqfb3paxppm5dnj/app.bsky.feed.post/3llf7t6vthk2d