Paroxysmal Nocturnal Hemoglobinuria (PNH) - Hemolytic Anemia - Complement Alternative Pathway
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- เผยแพร่เมื่อ 14 ต.ค. 2024
- Paroxysmal Nocturnal Hemoglobinuria (PNH) - Hemolytic Anemia - Complement Alternative Pathway
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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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imagine even after three years of sharing this videos, still students benefit you video....جزاك الله خيرا........from Somaliland
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Completely agree
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LOL! That’s funny! Thanks:)
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You made my day...Thanks a lot!
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!
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Congratulations! I am so happy for you!
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
Thank you 🙏
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!!!
My pleasure 😇
Good luck 🍀
Are you working on pnh trials in India?
You are extremely talented.
I can't thank you enough ❤️
It's like for the first time I have properly understood PNH
Thank you so much 😊
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
thanks👏
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
You’re such a delightful person 😁😁
Thank you so much!
I am so honored to help.
Can you please help me by sharing?
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
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?
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 :)
Your English is better than mine...Thank you...I will look it up!
What is msc?
Do you mean mesenchymal stromal cells?
You talk my language man: Clear and simple
Thanks 😊
Can you please help me by sharing?
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!
Awesome 👏
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Can you please help me by sharing?
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Grateful people are the best!
Great video. I needed rapid upskilling in PNH and this was super helpful - thank you.
My pleasure 😇
I have pnh and this is the best explanation I have watched . Thank you
Thank you 🙏
I wish you the best of luck!
This is a perfect tutorial you speak clearly
Very beneficial, and very comprehensive ( gives you the details in short ) . Excellent channel to follow , and for preparing exams . 👌
Thank you so much, brother!
Can you please help me by sharing?
Medicosis Perfectionalis sure
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love your voice and tone. very captivating lecture !
Thank you 😊
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💘💘💘💘💘💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙💙🙏🙏🙏🙏🙏🙏🙏🙏🙏🙏🙏
My pleasure 😇
Thank you 🙏
يعطيك العافيه .. شرح رائع حكيم ❤️🌹
Thanks 🙏
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
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Sir pnh ki dawa batao please sir
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.
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.
What’s the name of that drug?
@@MedicosisPerfectionalis it's acetylsalycilic acid (Aspirin), it's the same as syalic acid on platlets.
Wow sir, amazing as always👏👏🏋️♀️
May God bless you 🙏
Thank you so much, Natasha!
God bless you too!
Would you please help me by sharing?
Very nice. But I think you had missed anticoagulant as treatment option in case of thrombotic evidence.
True! I will mention that in my bleeding and coagulation playlist.
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).
Thanku 💙 The video really helped cleared my concepts. I’ve read PNH multiple times but this js the first time i actually understood it.
I am so happy to hear that! What an honor! Thanks for sharing this with me!
Nothing short of Brilliant...as usual..
Great work sir😎 ur such a life saviour
Thanks again
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Thank you...I wish you best of luck!
الله يوفقك❤
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Great explanation , thank you very much 🙏🏿🌺🌿
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Love the little funny things you say in the middle 😂♥️
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its actually CD59 which is potent inhibittor of C3 convertase
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?
I have PNH, but my daughter does not. It is not genetic.
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As long as it helps...I am satisfied :)
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Tq sir for making the things simple.which we found difficult😊
You’re very welcome 😊
Thank you so much.This is quite helpful
I am glad 😃
I always get the answer from your videos, thank you.
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U said there is no splenomegaly in iv hemolytic why here is and thanx for the amazing explanation from the best medicosis 😍????
There are exceptions to every rule.
Wow this is excellent. Thank you so much for such a clear explanation.
CD IN CD55 CD59 stands for complement defence
very, very good! clear and to the point, thank you
Thanks a ton!
Hi medicosis perfectionalis, I love the structure of your videos and the way you explain. Do you have any videos on the classical pathway of complement cascade?
Thanks a ton, dear! Unfortunately, I don’t!
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?
Thank you so much Sir. May I please ask, why is there splenomegaly if the hemolysis takes place intravascular?
Thanks in advance.
I came to ask the same question
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My pleasure 😇...Welcome to Medicosis :)
thanks for the video, it helped alot
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Thank for ur good explanation
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Can you please tell me that the content you are creating is made from standard text books . Can we use this values for indian pg entrance exam ? Thanks in advance
That was beyond wonderful
Thank you 🙏
Thank you
Also usually no spleenomegaly as hemolysis is intravascular
I Love you From Pakistan.dr Raheela
Thank you!
Wow so useful. Thank u for making this video 👍🏻
Learning something new today 😁
Great! Thank you for watching!
amazing.. concept clearing breakdown of information!
Thanks a million for your kind words! I am so delighted you liked it!
You explain amazingly. Thank you.
What a nice comment! Thanks a ton!
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Splendid🖤🔥
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That was fast n informative tysm
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wow amazing video !! thanksss
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.
Doesn't you haemoglobin reduce? What you will do to keep your Haemoglobin stable?
@@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.
U man earned a subscriber 😉
Welcome aboard, brother!
"thrombosis is thought to be caused by a lack of CD59 on platelet membranes predisposing to platelet aggregation"
In this video Every thing about PNH was so simplified that I didn't scape reading about it for the first time thanks a lot. There is some thing at the end of the video that talks about Low level of CD55 and CD59 in flowcytometry. If the defect is in GPI gene and it can't bind to CD55 &CD59 why these complements decrease while they are not used and their level should increase in blood😐?
It’s not that they are there and not being used...They are genetically deficient...It’s like the difference between type 1 Diabetes (no insulin), and type 2 Diabetes (insulin isn’t being used)...So, PNH in that sense is similar to type 1 DM...Hope it helps!
Medicosis Perfectionalis thanks I got it.👍
My pleasure! Take care!
Amazing..
Thank you!
Nice video, but u didn't explain why it's NOCTURNAL ??
Tnx a Bunch
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Thank you for your excellent video
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Superb
Hi Dr.
There is no splenomegaly in PNH because the hemolysis occurs intravasculr not extravascular.I need to explanation for that because u r say it cause splenomegaly(please make the answer by references).Thanx from my heart 💓
Hey 👋 Ahmed.
Good question!
Reference:
Harrison’s internal Medicine, 20th edition, chapter 62, page 415, there is a table titled “Diseases associated with splenomegaly”...PNH is there 🤗
So, there is splenomegaly in PNH.
Just because it’s “predominantly” intravascular doesn’t mean that splenomegaly is impossible.
Good luck!
@@MedicosisPerfectionalis
Great info. I found it, thanx for your help💓
You’re welcome
Thank you for the great lecture .
My pleasure 😇
Question plz .. why it cause splenomegaly altought it is intravscular hemolysis ?
You’re right, but don’t forget that Budd-Chiari can have splenomegaly!
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Thanks bro u really safe me for my exams.☺☺☺
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 😃😃
I have already share it .medicosis perfectionalis.....
Thank you :)
Well you said its acquired but its a autosomal recessive diesease as written in robins basis pathology
According to Harrison’s internal Medicine (19th edition, page 660): PNH is an acquired disorder.
Where did you find it in Robbin’s?
Robbin’s basic pathology (9th edition, page 51): “PNH is an acquired deficiency of DAF...”
@@MedicosisPerfectionalis yes its page number 421 of pocket robins....it says paroxysmal nocturnal hemoglobinuria (pnh) is a rare x linked hemolytic disease resulting from acquired mutation in PIGA which confused me.
What’s the year of the publication?
Good job👍👍👍
Thanks 😃
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Sure!
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so nice
Thanks 🙏
New follower. Thank youuu
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Thank you thank you thank you thank you
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in beginning you are quiet and in the end you are fast 😁😘
Haha
Thanks a ton sir🙏🏼🙏🏽
This was very very helpful thank you
Don’t mention it! My pleasure! 😁
I am in query some literature said DAT is positive in PNH but in some it's negative..😢
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amazing man, thank you
My pleasure 😇