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

Paroxysmal Nocturnal Hemoglobinuria (PNH) - Hemolytic Anemia - Complement Alternative Pathway
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►👨‍🏫💊Antibiotics Lectures: www.medicosisp... ... Check out my brand new "Electrolytes" course at www.medicosisp... and use the PROMO code: ELECTROLYTES50 to get a 50% discount. Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired defect in the PIGA gene, deficiency of CD55 and CD59 is evident...The alternative complement pathway is activated attacking the red blood cells causing intravascular hemolysis.
Paroxysmal nocturnal hemoglobinuria is an intrinsic, intravascular, acquired hemolytic anemia.
Complications of paroxysmal nocturnal hemoglobinuria (PNH): iron deficiency anemia (IDA), acute myeloid leukemia (AML), and thromboses (the most common cause of death).
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[@MedicosisPerfectionalis]

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[@MedicosisPerfectionalis]

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[@MedicosisPerfectionalis]

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[@MedicosisPerfectionalis]

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[@MedicosisPerfectionalis]

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[@MedicosisPerfectionalis]

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[@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]

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[@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 😊

[@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]

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[@MedicosisPerfectionalis]

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[@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]

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[@elpedro812]

You talk my language man: Clear and simple

[@MedicosisPerfectionalis]

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[@bardacha100]

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[@MedicosisPerfectionalis]

Thank you so much 😊

[@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?

[@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?

[@user-if5tq9pi3w]

This is a perfect tutorial you speak clearly

[@rushi_desai]

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

[@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?

[@HexBore13]

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

[@MedicosisPerfectionalis]

My pleasure 😇

[@obadaalqudah9270]

يعطيك العافيه .. شرح رائع حكيم ⁦❤️⁩🌹

[@MedicosisPerfectionalis]

Thanks 🙏

[@veronicagomez4457]

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

[@MedicosisPerfectionalis]

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[@MedicosisPerfectionalis]

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[@MedicosisPerfectionalis]

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[@muhammadal-roumani8962]

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[@MedicosisPerfectionalis]

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[@MedicosisPerfectionalis]

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[@MedicosisPerfectionalis]

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[@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]

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[@MedicosisPerfectionalis]

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[@MedicosisPerfectionalis]

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[@user-up4eg4uh7i]

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[@MedicosisPerfectionalis]

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[@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

[@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.

[@Heba-je1en]

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[@MedicosisPerfectionalis]

My pleasure 😇

[@jagadeeshj1645]

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[@MedicosisPerfectionalis]

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[@aroor1785]

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[@MedicosisPerfectionalis]

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[@manuelworth7007]

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

[@MedicosisPerfectionalis]

Thanks a ton!

[@sokhengung7983]

Thank for ur good explanation

[@MedicosisPerfectionalis]

My pleasure 😇

[@darsama2655]

الله يوفقك❤

[@MedicosisPerfectionalis]

🙏🙏

[@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).

[@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.

[@Saaraelamin]

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

[@MedicosisPerfectionalis]

My pleasure 😇

[@drmohammedhamzah1880]

CD IN CD55 CD59 stands for complement defence

[@jjmifsud3618]

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

[@abhineshramesh7400]

its actually CD59 which is potent inhibittor of C3 convertase

[@mlt2year]

Amazing..

[@MedicosisPerfectionalis]

Thank you!

[@drmohammedhamzah1880]

Also usually no spleenomegaly as hemolysis is intravascular

[@rayayalmaz9403]

Tnx a Bunch

[@MedicosisPerfectionalis]

My pleasure 😇

[@nadia6334]

wow amazing video !! thanksss

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Thank you!!!!!!😊

[@MedicosisPerfectionalis]

My pleasure!

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[@MedicosisPerfectionalis]

Thank you so much 😊

[@dr.ahmdooz]

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

[@MedicosisPerfectionalis]

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[@MedicosisPerfectionalis]

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[@MedicosisPerfectionalis]

My pleasure 😇...Welcome to Medicosis :)

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Splendid🖤🔥

[@MedicosisPerfectionalis]

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Superb

[@karunasethi6884]

You explain amazingly. Thank you.

[@MedicosisPerfectionalis]

What a nice comment! Thanks a ton!

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

[@MedicosisPerfectionalis]

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

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Thankyou, you are amazing.

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

[@MedicosisPerfectionalis]

Great! Thank you for watching!

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

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Thank you thank you thank you thank you

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

[@MedicosisPerfectionalis]

You are welcome :)

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U man earned a subscriber 😉

[@MedicosisPerfectionalis]

Welcome aboard, brother!

[@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.

[@bushraliaqat9522]

U r so intelligent

[@MedicosisPerfectionalis]

Thanks, dear :)

[@srinivasaraosirasapalli5104]

so nice

[@MedicosisPerfectionalis]

Thanks 🙏

[@dom4638]

great job!! ty!

[@akygadkar13]

amazing.. concept clearing breakdown of information!

[@MedicosisPerfectionalis]

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

[@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.

[@naglaafarrag5646]

Thank you for your excellent video

[@MedicosisPerfectionalis]

My pleasure 😇

[@indefat]

"thrombosis is thought to be caused by a lack of CD59 on platelet membranes predisposing to platelet aggregation"

[@rantaz07]

amazing man, thank you

[@MedicosisPerfectionalis]

My pleasure 😇

[@nabilamuruthi9661]

Never disappoints

[@MedicosisPerfectionalis]

Thank you 🙏

[@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

[@user-yz4mo4vi6q]

Why there is esophagus spasm whats the mechanism?

[@iamyanez6553]

New follower. Thank youuu

[@MedicosisPerfectionalis]

My pleasure 😇

[@medicallover8285]

Why bone suppression occure in PNH mechaniam

[@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?

[@manarsmaan2624]

Thank you for the great lecture .

[@MedicosisPerfectionalis]

My pleasure 😇

[@drag3nlove249]

Good job👍👍👍

[@MedicosisPerfectionalis]

Thanks 😃

[@sluggy-1433]

This was very very helpful thank you

[@MedicosisPerfectionalis]

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Amazing! Thank you so much

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thank you

[@MedicosisPerfectionalis]

You’re always welcome 🙏

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

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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 😃😃

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

[@MedicosisPerfectionalis]

Thank you :)

[@doomsdayknight2229]

Nailed it! 🔨 💯

[@MedicosisPerfectionalis]

Thanks!

[@himel7921]

brilliant

[@MedicosisPerfectionalis]

Thank you 😊

[@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

[@gff10038]

Just Wooooow!! Thanks a lot!!

[@MedicosisPerfectionalis]

My pleasure 😇

[@gff10038]

@@MedicosisPerfectionalis reticulocytes are immature RBC😊🤓

[@athulyavs6735]

Why is glucocorticoid contraindicated in PNH

[@srinivasaraosirasapalli5104]

excellent

[@MedicosisPerfectionalis]

Thank you...Appreciate it

[@ahmadhammad3769]

Why glucocirticoids are contraindicated?

[@darrenrodrigues5962]

A doubt how do thrombocytopenia and thrombosis coexist?

[@lc9594]

I've had pnh since 14, 40 now. I do not or have ever taken anything for it... usually I forget I even have it, until I see the blood in my urine which has happened twice now 4 years apart.

[@sachinp1950]

Doesn't you haemoglobin reduce? What you will do to keep your Haemoglobin stable?

[@lc9594]

@@sachinp1950 I use reiki, and herbal medicine. I do the best that I can to live a healthy life. I eat organic as much as possible, I eliminate Fluoride, ect.