Paroxysmal Nocturnal Hemoglobinuria (PNH) - Hemolytic Anemia - Complement Alternative Pathway

[@MedicosisPerfectionalis]

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[@asdssa2714]

imagine even after three years of sharing this videos, still students benefit you video....جزاك الله خيرا........from Somaliland

[@MedicosisPerfectionalis]

Thank you so much 😊

[@robertsarhanmd6433]

I must say this was an Excellent tutorial, you spoke at a perfect speed you were clear , you are an Excellent Professor

[@MedicosisPerfectionalis]

Thank you so much 😊

[@camnguyen6838]

Completely agree with your comment.

[@mrsamandabernier]

Completely agree

[@atikahkhaulah311]

"did u understand the thing?" "of course not" I was like "ok, dude u got me 😅😂"

[@MedicosisPerfectionalis]

LOL! That’s funny! Thanks:)

[@DrElla-vm9mo]

Unbelievable how you put all textbooks information in a simple 11 minute video Thanx for you, it help us a lot ❤️

[@iamgopz]

2:28 Complement System 3:04 Alternative pathway of complement system 4:25 Complement Regulatory proteins 5:59 Overview of PNH 7:54 Clinical Manifestations 9:05 Diagnosis 10:46 Treatment

[@asdssa2714]

thanks👏

[@amnahah5012]

The content you have is so simple and comprehensive. Thank you for saving lives.

[@MedicosisPerfectionalis]

You made my day...Thanks a lot!

[@MedicosisPerfectionalis]

🩸 Hematology Playlist: kzbin.info/aero/PLYcLrRDaR8_eoNz6dxXolh1XMEietcniU&si=tomFsUjAgOKhOJVq

[@yannur6841]

Sir you just helped me pass a hematology quiz based on material from Harrison!!! this way of studying is so much more relatable and fun and USEFUL(!!!) than reading a book!! Thank you so much for all of your blessed work, may G-d almighty bless you to keep creating and teaching and helping millions!

[@MedicosisPerfectionalis]

Thank you so much 😊 Congratulations! I am so happy for you!

[@Heartenstein]

As crazy as it sounds, since I discovered your videos, I'm excited every morning to start studying for my residency exam! Thank you for the work and dedication!

[@MedicosisPerfectionalis]

Wow 🤩 Good luck 🍀

[@orezimenaomokiti1300]

I've been trying to read this topic for weeks but each time I looked at my lecture not I got frustrated and stopped trying to get the pathogenesis. This video is a life saver

[@MedicosisPerfectionalis]

Thank you 🙏

[@hakeemahali8813]

I won't stop saying: YOU ARE THE BEST!

[@MedicosisPerfectionalis]

Thank you so much 😊 You’re the best! Grateful people are the best!

[@MedicosisPerfectionalis]

💉 📜 50 hematology cases are waiting, as well as the answer to today's question...Go to www.patreon.com/medicosis/ 😅 If you like mnemonics, try Picmonic: www.picmonic.com/referrals/JK55NQXQQB9JLC119 👩‍💻Sign up for Dropbox here: db.tt/IIlB8ejxWe 📕📗 Books that I recommend, www.amazon.com/shop/medicosisperfectionalis

[@palaksingh7478]

Sir pnh ki dawa batao please sir

[@raadeyah7004]

You are extremely talented. I can't thank you enough ❤️ It's like for the first time I have properly understood PNH

[@MedicosisPerfectionalis]

Thank you so much 😊

[@bardacha100]

This guy is a genius. Best professor ever !

[@MedicosisPerfectionalis]

Thank you so much 😊

[@elpedro812]

You talk my language man: Clear and simple

[@MedicosisPerfectionalis]

Thanks 😊 Can you please help me by sharing?

[@dge.]

I found you again!! ONCE AGAIN I WOULD LIKE TO THANK YOU FOR PROVIDING US WITH SUCH clear and concise videos. if another person did this video i’m 100% sure I’d end up falling asleep. PLEASE UPLOAD MORE! - a cramming 2nd year medical student

[@MedicosisPerfectionalis]

You’re such a delightful person 😁😁 Thank you so much! I am so honored to help. Can you please help me by sharing?

[@Pankaj-045]

I am working on a PNH clinical trial and have no clinical background, this is such a wonderful video is for understanding about this. Thank a lot!!!

[@MedicosisPerfectionalis]

My pleasure 😇 Good luck 🍀

[@rohitwaldia5892]

Are you working on pnh trials in India?

[@jayamanoj4277]

Thanks so much! This saved a ton of time trying to read through different review articles and MOAs. Very clear, conscise, and yet covers all important points!

[@MedicosisPerfectionalis]

Awesome 👏 Thank you so much 😊 Can you please help me by sharing?

[@veronicagomez4457]

I have pnh and this is the best explanation I have watched . Thank you

[@MedicosisPerfectionalis]

Thank you 🙏 I wish you the best of luck!

[@Rushi_Desai]

i can't stop my tears , thank you thank you sir🥺🥺🥺🥺

[@cs8844]

I'd like to add something: 1) It's nocturnal also because at night T° is lower and C5 tends to form C5 convertase better (also for lower pH at night) 2) The reason why those MSC are subject to positive selection pressure (probably) is that the patient develops an autoimmune disease against some GPI-proteins. You need to explain why mutated-MSC are selected since this is an acquired mutation and that's what my prof. said

[@MedicosisPerfectionalis]

Thanks for the comment... I have checked Harrison‘s internal medicine textbook, and there is no mention of any autoimmune disease against the GPI protein. It says that it’s merely a deficiency of the GPI, caused by a mutation of an X-linked gene called PIG-A gene, and that this mutation is different from one patient to another due to the fact that this mutation is not inherited, and it’s acquired during hematopoiesis. So, do you have a reference that I can look at?

[@cs8844]

Yes, this theory is reported on Robbins and Cotran Pathologic Basis of Disease (at least in the italian version that i use)! My professor said that there must be something that induces a positive selection pressure (because otherwise the problem would be isolated to a single MSC) and this something is probably an autoimmune disease against some GPI proteins (so mutaded MSC are ok and normal are attacked). He also said that this theory helps to understand why PNH is common in patients with bone marrow aplasia (that has autoimmune bases). Hope my english is understandable, thank you for your videos :)

[@MedicosisPerfectionalis]

Your English is better than mine...Thank you...I will look it up!

[@medicosisshorts5604]

What is msc?

[@medicosisshorts5604]

Do you mean mesenchymal stromal cells?

[@obadaalqudah9270]

يعطيك العافيه .. شرح رائع حكيم ⁦❤️⁩🌹

[@MedicosisPerfectionalis]

Thanks 🙏

[@HexBore13]

Great video. I needed rapid upskilling in PNH and this was super helpful - thank you.

[@MedicosisPerfectionalis]

My pleasure 😇

[@saky9436]

love your voice and tone. very captivating lecture !

[@MedicosisPerfectionalis]

Thank you 😊

[@KhaysanSalis]

This is a perfect tutorial you speak clearly

[@mjk9152]

You always have great merit for my life and my dream. I will pray to God to protect you and help you. Thank you from the heart💘💘💘💘💘💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙🙏🙏🙏🙏🙏🙏🙏🙏🙏🙏🙏

[@MedicosisPerfectionalis]

My pleasure 😇 Thank you 🙏

[@Budo99]

I love your incredibly dry sense of humor. It helps!

[@MedicosisPerfectionalis]

As long as it helps...I am satisfied :)

[@Cat-lm5fq]

Great work sir😎 ur such a life saviour Thanks again

[@MedicosisPerfectionalis]

Thank you so much ☺️

[@lucilaalvarez4095]

Nothing short of Brilliant...as usual..

[@muhammadal-roumani8962]

Very beneficial, and very comprehensive ( gives you the details in short ) . Excellent channel to follow , and for preparing exams . 👌

[@MedicosisPerfectionalis]

Thank you so much, brother! Can you please help me by sharing?

[@muhammadal-roumani8962]

Medicosis Perfectionalis sure

[@natasha1394]

Wow sir, amazing as always👏👏🏋️‍♀️ May God bless you 🙏

[@MedicosisPerfectionalis]

Thank you so much, Natasha! God bless you too! Would you please help me by sharing?

[@اميراحمدعبدالكاظمحسن]

You allways brilliant 😍🥰😇😊❤❤❤❤

[@فاطمةالزهراء-ن9ظ5ل]

Great explanation , thank you very much 🙏🏿🌺🌿

[@MedicosisPerfectionalis]

My pleasure!

[@Baldwinthefourth-1176]

Absolutely brilliant

[@MedicosisPerfectionalis]

Thank you!

[@umbrella2056]

Thanku 💙 The video really helped cleared my concepts. I’ve read PNH multiple times but this js the first time i actually understood it.

[@MedicosisPerfectionalis]

I am so happy to hear that! What an honor! Thanks for sharing this with me!

[@renassalahudden9520]

thank you so much tomorrow i have an exam and ur just a hero saved me ❤️

[@MedicosisPerfectionalis]

Thank you...I wish you best of luck!

[@saraalmujhem1006]

oh .. a great great channel... luv u guys and thank u 🕯📚

[@MedicosisPerfectionalis]

My pleasure 😇...Welcome to Medicosis :)

[@darsama2655]

الله يوفقك❤

[@MedicosisPerfectionalis]

🙏🙏

[@ahmadaljazeeri9287]

Well done man! Another amazing enjoyable video 😄

[@MedicosisPerfectionalis]

Thank you so much 😊

[@Saaraelamin]

I always get the answer from your videos, thank you.

[@MedicosisPerfectionalis]

My pleasure 😇

[@manuelworth7007]

very, very good! clear and to the point, thank you

[@MedicosisPerfectionalis]

Thanks a ton!

[@keerthivasanjRapper]

This is amazing , keep em coming brother! 🌸🔥 Love the little funny things you say in the middle 😂♥️

[@chimeziembah3099]

Thank you so much.This is quite helpful

[@MedicosisPerfectionalis]

I am glad 😃

[@umarkhader7313]

U man earned a subscriber 😉

[@MedicosisPerfectionalis]

Welcome aboard, brother!

[@rizwanarehman4464]

Hi. All your videos are just amazing. I'm astounded , how can a person explain a complicated topic in such a detail in such a short time 😍❤. Thanks ❤. You deserve more subscribers 😌

[@MedicosisPerfectionalis]

Thank you so much 😊

[@jagadeeshj1645]

Tq sir for making the things simple.which we found difficult😊

[@MedicosisPerfectionalis]

You’re very welcome 😊

[@rayayalmaz9403]

Our Pr of hematology that i respect soo much for the informations that he gave us concerning coagulation, said that the outside membrane of platlets is charged negatively cause it has syalic acid, it's an anti-aggregant, this is why we give it as a drug to increase repulsion. When a platlet ruptures, it shows it's positive charge so the other platlets aggragate to it.

[@MedicosisPerfectionalis]

What’s the name of that drug?

[@rayayalmaz9403]

@@MedicosisPerfectionalis it's acetylsalycilic acid (Aspirin), it's the same as syalic acid on platlets.

[@abdullahahmed8621]

Splendid🖤🔥

[@MedicosisPerfectionalis]

Thank you so much 😊

[@lifeisagift.cherisheverymoment]

I was diagnosed with PNH in 2018. Is there an online support community where I can learn from and relate to others with PNH? Knowing the mechanics of PNH is enlightening, but tells me nothing about what I am experiencing and how it relates to others with PNH. Thank you.

[@karunasethi6884]

You explain amazingly. Thank you.

[@MedicosisPerfectionalis]

What a nice comment! Thanks a ton!

[@jjmifsud3618]

Wow this is excellent. Thank you so much for such a clear explanation.

[@dr.md.imrulkaes9793]

Very nice. But I think you had missed anticoagulant as treatment option in case of thrombotic evidence.

[@MedicosisPerfectionalis]

True! I will mention that in my bleeding and coagulation playlist.

[@dr.md.imrulkaes9793]

Yes that is true. Thromboembolic events are the leading cause of morbidity and mortality in PNH. Acute thrombotic events require anticoagulantion with heparin ( if no absolute contraindication for heparin) ; and anticoagulant should continue with eculizumab ( it self reduces risk of thrombotic events) in those patient. Not only that it also recommended that patient with more than 50%-60% GPI AP deficient PMNs be offered prophylactic anticoagulation. You can go through " Update on the diagnosis and management of paroxysmal nocturnal hemoglobinuria" - Hematology 2016. It is mentioned in Text books also, " There is now increasing confidence that the catastrophic thrombotic complications of PNH can much more successfully managed with combination of eculizumab and anticoagulant."- Postgraduate Haematology 7th edition (By A Victor Hoffbrand).

[@Hebayousef29]

thanks for the video, it helped alot

[@MedicosisPerfectionalis]

My pleasure 😇

[@sokhengung7983]

Thank for ur good explanation

[@MedicosisPerfectionalis]

My pleasure 😇

[@HammadKhan-zw8mm]

Thanks bro u really safe me for my exams.☺☺☺

[@MedicosisPerfectionalis]

You’re so welcome...Good luck with your exam...When you do well on your exam, please consider sharing these videos with your friends...Thanks a million 😃😃

[@HammadKhan-zw8mm]

I have already share it .medicosis perfectionalis.....

[@MedicosisPerfectionalis]

Thank you :)

[@aroor1785]

That was beyond wonderful

[@MedicosisPerfectionalis]

Thank you 🙏

[@akygadkar13]

amazing.. concept clearing breakdown of information!

[@MedicosisPerfectionalis]

Thanks a million for your kind words! I am so delighted you liked it!

[@sitarasarfaraziqbal4789]

Hello Sir,Can you please explain why glucocorticoids are contraindicated in PNH?THANKS And your work is AMAZING as usual,keep it up.STAY BLESSED.AMEEN

[@drag3nlove249]

Good job👍👍👍

[@MedicosisPerfectionalis]

Thanks 😃

[@Germatti13489]

My daughter was diagnosed last year with pnh but her cousin, my twin brother's son, was diagnosed with the familial cousin of the disease called spherocytosis at the age of 4. Are you sure that this is an acquired autoimmune disease, not a genetic disease?

[@bmrcustoms2969]

I have PNH, but my daughter does not. It is not genetic.

[@genqsolusi5399]

Wow so useful. Thank u for making this video 👍🏻 Learning something new today 😁

[@MedicosisPerfectionalis]

Great! Thank you for watching!

[@ishaanmishra547]

Thanks a ton sir🙏🏼🙏🏽

[@RaheelaSarwat]

I Love you From Pakistan.dr Raheela

[@MedicosisPerfectionalis]

Thank you!

[@malathabushanab2222]

That was fast n informative tysm

[@MedicosisPerfectionalis]

My pleasure 😇

[@dr.ahmdooz]

in beginning you are quiet and in the end you are fast 😁😘

[@MedicosisPerfectionalis]

Haha

[@naglaafarrag5646]

Thank you for your excellent video

[@MedicosisPerfectionalis]

My pleasure 😇

[@doomsdayknight2229]

Nailed it! 🔨 💯

[@MedicosisPerfectionalis]

Thanks!

[@LD-ky8bo]

Thank you!!!!!!😊

[@MedicosisPerfectionalis]

My pleasure!

[@manarsmaan2624]

Thank you for the great lecture .

[@MedicosisPerfectionalis]

My pleasure 😇

[@sluggy-1433]

This was very very helpful thank you

[@MedicosisPerfectionalis]

Don’t mention it! My pleasure! 😁

[@melodykimbrough713]

Is it called paroxysmal because the hemoglobin becomes more concentrated at night or the because of the pH change with hypoventilation when we sleep? Or both?

[@fatimaaamir214]

Excellent

[@MedicosisPerfectionalis]

Thanks!

[@MK-bi8xl]

dsy ro ro shuru ye kichway ghndy mata dy khob rawasto 🙂

[@abhineshramesh7400]

its actually CD59 which is potent inhibittor of C3 convertase

[@amaniewallace7021]

Excellent!!!!!!!! Thank you!!!!!!!!

[@MedicosisPerfectionalis]

You are welcome :)

[@nadia6334]

wow amazing video !! thanksss

[@ahmadhammad3769]

U said there is no splenomegaly in iv hemolytic why here is and thanx for the amazing explanation from the best medicosis 😍????

[@MedicosisPerfectionalis]

There are exceptions to every rule.

[@lebocarolinenkosi5533]

Thank you so much Sir. May I please ask, why is there splenomegaly if the hemolysis takes place intravascular? Thanks in advance.

[@nishajanagal940]

I came to ask the same question

[@Alahmora]

Why is glucocorticoid contraindicated in PNH

[@viv3081]

wow man...amazing

[@MedicosisPerfectionalis]

Great...I am so glad you liked it!

[@viv3081]

Medicosis Perfectionalis I used your videos last semester and they really helped me understand anemias. Please do some videos on myeloproliferative, lymphoproliferative diseases and leukemias, they will be really really appreciated! Thanks again for all your awesome work!

[@MedicosisPerfectionalis]

Of Course...All of these topics are coming after anemia....Please share! It really helps me...Good luck...from the bottom of my heart :)

[@viv3081]

Medicosis Perfectionalis will do! Thanks a million!

[@MedicosisPerfectionalis]

Thank YOU

[@iamyanez6553]

New follower. Thank youuu

[@MedicosisPerfectionalis]

My pleasure 😇

[@rayayalmaz9403]

Tnx a Bunch

[@MedicosisPerfectionalis]

My pleasure 😇

[@ahmadhammad3769]

Why glucocirticoids are contraindicated?

[@VicbengtPL]

Amazing! Thank you so much

[@shibbirahmedshohag1659]

So,,is phn is curable??i cousin in bangladesh is suffering from phn.but the doctor said it do not have any permanent solution except stem cell operation. But it is too costly and have a huge risk.is there in permanent solution to cure by taking any type of medicine??

[@rantaz07]

amazing man, thank you

[@MedicosisPerfectionalis]

My pleasure 😇

[@darrenrodrigues5962]

A doubt how do thrombocytopenia and thrombosis coexist?

[@drmohammedhamzah1880]

CD IN CD55 CD59 stands for complement defence

[@دكتورمحمدضياءمحمدأحمد]

Why there is esophagus spasm whats the mechanism?

[@bushraliaqat9522]

U r so intelligent

[@MedicosisPerfectionalis]

Thanks, dear :)

[@drmohammedhamzah1880]

Also usually no spleenomegaly as hemolysis is intravascular

[@ashaltimalsina8660]

Thankyou, you are amazing.

[@mlt2year]

Amazing..

[@MedicosisPerfectionalis]

Thank you!

[@alimeran7442]

Question plz .. why it cause splenomegaly altought it is intravscular hemolysis ?

[@MedicosisPerfectionalis]

You’re right, but don’t forget that Budd-Chiari can have splenomegaly!

[@alimeran7442]

❤

[@gff10038]

Just Wooooow!! Thanks a lot!!

[@MedicosisPerfectionalis]

My pleasure 😇

[@gff10038]

@@MedicosisPerfectionalis reticulocytes are immature RBC😊🤓

[@meissharing]

I am in query some literature said DAT is positive in PNH but in some it's negative..😢

[@medicallover8285]

Why bone suppression occure in PNH mechaniam

[@nabilamuruthi9661]

Never disappoints

[@MedicosisPerfectionalis]

Thank you 🙏

[@alprazolamrx9710]

Nice video, but u didn't explain why it's NOCTURNAL ??