PNH: When blood machinery goes wrong

In paroxysmal nocturnal haemoglobinuria (PNH), the immune system mistakes red blood cells for dangerous invaders and attacks them. This life-threatening disease is triggered by a fault in the body's blood-making machinery, and although therapies are available, researchers are still working on improving treatments.
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[@lilacspring2556]

This level of communication is so inspiring

[@djpupsik98]

what did you expect from cells that cannot see/hear/speak in common manner? only chemicals, only hardcore

[@Tempst]

One of the best animations from nature video

[@revan012]

Should have also added that Paroxysmal Nocturnal Hemoglobinuria can correct itself. I was diagnosed with 80% clone size now at 0.2% clone size. I am very lucky.

[@leilaali7896]

Can you please share if you have followed any diet, medication. Also, were you following any treatment? What were your symptoms? Thank you

[@revan012]

​@@leilaali7896 I was diagnosed with PNH back in 2004 with an 80% clone size when I was thirteen, back then the only treatment to "cure" PNH was a bone marrow transplant. However, the benefits did not outweigh the risks. the only medication I was put on was iron tablets, warfarin and folic acid. Before my diagnosis I was very active and fit, I played football, went running and trained in martial arts (Judo.) I had to stop after my diagnosis due to being put onto warfarin, contact sports were a risk. I did however, continue to run and started weight training (only light not extreme again due to warfarin.) My diet was manly white meat with the recommend daily intake of fruit/veg. I very rarely ate red meat. My symptoms considering I had 80% abnormality were mild. At times I could feel quite lethargic even with plenty of sleep, this was mostly after being unwell i.e common cold flu etc. Recovery from a cold/flu took longer than most people two three weeks. The main symptom that led to a PNH diagnosis was after a bout of very dark urine (deep brown.) caused by haemolysis. Even with the destruction of red blood cells I did not need to have blood transfusions. I was already being seen by a haematology specialist due to low red blood platelets however, I was about to be discharged from their care due to this returning to normal levels when the PNH symptom occurred. The main treatment for symptomatic PNH is eculizumab (Soliris) with also ravulizumab becoming available on the NHS. The cost on average for both is still exceedingly high so I do not know if it is covered by insurance. luckily I am in the UK so both are covered by the NHS.

[@nayanc6533]

I also need this info 😭

[@hadihasan4661]

How , can you tell please.

[@DefinitelyNotaFurrySpy-zq2zs]

Blood cell: forgets to put on name tag Immune system: so you have chosen…death

[@neymeravenir6126]

No that only thatsunlucky

[@filipthunell8631]

immune system: sees a cell without a name tag also immune system: ALARM ALARM ALARM

[@treesimp6686]

so immune cells to blood cells without tag is like a teacher in kindergarden if you dont wear your tag.

[@sinkrada419]

Blood cell: forgets to put on name tag Immune system: THERE IS A RED SPY IN THE BASE

[@WasabiSniffer]

@@sinkrada419 WE NEED TO PROTECT THE BRIEFCASE!

[@applea8253]

I DONT EVEN NEED TO LISTEN THE ANIMATION SAYS IT ALL. THANK YOU

[@gurkiratsingh4157]

That part where complement system came in and MAC was formed was just awesome Cleared my concepts

[@enderman700]

There is more structure in yourself but u are the main leader of your body.

[@nerdicperson6235]

Its always fascinating how well the immune system can function in a body, and how easily the immune system can turn against the body.

[@pribarros1681]

Immune System when a minor issue happens in your body: "you know when a kid screams? yea that"

[@65snehakothapalli98]

Very WONDERFUL and intriguing level of explanation and visuals combined, simply awesome..keep doing more

[@MedQ7]

Fantastic animation 👍 with beautiful explanation…

[@dncmi1436]

1:52 look at the bad boi behind.....the expression kills me 😂😂😂

[@drilldoggoboi5618]

That's cells

[@sucsotheautdam1311]

@@drilldoggoboi5618 That's bacterial

[@sucsotheautdam1311]

A green boi

[@alijasim8567]

Great explanation, amazing job, thank you so much👌👌👌❤️❤️❤️

[@tysoncook5152]

Very well made!

[@gamerx112]

great. just yet another thing to be thinking about.

[@godzuky2743]

KZbin: "COVID-19 Get the latest information from the CDC about COVID-19." Literally anyone with eyes and ears watching this: "Yes thank you for telling me something completely irrelevant to the video I am watching explaining a blood disorder."

[@hernanhernandez6771]

I have this and I’m so nervous I’m afraid of a bad outcome

[@stevesabba2379]

Doing my ULTRAKILL homework dont mind me

[@هبهحسنعبدقحطان]

The most interesting video I have ever seen 🎉❤

[@WhomeCouldntbe1]

2:21 rip cell

[@mariusphilippebulawan4671]

Blood : nope hurt Antibody : touch hand Complement : what is your problem Antibody : ???

[@canavirichurakarenanahi9670]

beautiful

[@fantasyromance7560]

Scary.

[@ezgi8684]

harika bi video

[@AverageViet]

Tell the brain for not fixing the machines

[@uncredited_user2602]

0:17 iPhone alarm 😳😤😤😳😳😩

[@trashyCorn.12]

2:23 if only the incpetor cells could see also I probably spelled insceptor wrong

[@haiaarshid3781]

wow love it

[@mrhoho]

Good day. mate.

[@Ceres4S2D1]

This video has nothing to do with COVID-19, KZbin...

[@rohaizaismail2037]

😢 TX

[@skoience5910]

that like to dislike ratio tho

[@phantram6626]

PNH

[@МусабегХидиров]

♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡♡

[@raedwald-red]

'Paroxy-S-mal', not 'paroxymal'. Good video, but you really ought to coach your narrator in pronunciation of medical terminology.

[@humaidyzunuraini4854]

Red blood cell: ×_× White blood cell: >:>(

[@Englishreadingtips]

0:19

[@pikapixel4219]

bruh I thought the thumbnail was a geometry dash level

[@KJKP]

The good money says that one day, this problem will be resolved by improving the health of gut flora.

[@japooskas]

Problem is with a clonal line of blood stem cells genetics .. almost impossible that gut flora is involved

[@AtlasReburdened]

@@japooskas Yeah, perhaps not through the microbiome health directly, but there may be some group of compounds that alter the epigenetics to favor a similar gene to be expressed instead, which is inferior to the fully functional modern one, but better than the currently expressed and faulty modern one. There's a lot of secret utility hiding in the "unused" bits of our DNA.

[@yukwanchen5937]

???????????????

[@ภควัตพันธุ์สว่าง]

, Lol

[@OvertakeYou]

Marvellous animation, pretty accurrate.

[@bluelightstudios6191]

Blood cells: don't have tags Immune system: slaughters thousands of blood cells The Fred flintstone vita gummies I ate when I was 5: "Alright, someone better be explaining to what the problem is!!!"

[@AphidOs]

As someone who was diagnosed with pnh last year. First off these comments are very funny. 2nd I had to do transplant, my transplant failed but I’m doing a 2nd transplant. I no longer have pnh but the pnh caused severe aplastic anemia which is hard to get rid of.

[@Syxany]

I wish you luck, I'll be praying for you.

[@hadihasan4661]

You”ll be fine soon .

[@professionalnoob5474]

2:28 Pov: friendly fire is on

[@comfynarwhal]

This reminds me of something else I was watching in yt shorts and how they made something so boring like science (at least too me) a lot more understandable and fun to learn with a cute animation like this that gives you an idea on how it works by using more understandable stuff like the certificate on the blood cells

[@manel8843]

Kurzgesagt is kinda like this way,i really recommend kurzgesagt channel!

[@orenkrimchansky]

beautiful!

[@paulborisiv519]

So the Immune System basically miss-identifies it as a Cancer Cell, interesting, at first, going by what i saw in the initial part of the video, i started assuming it was a problem during the T-Cell's Activation, that for some reason faulty T-Cells were not being killed and had "graduated" despite their bad-readings, way to prove me wrong, still kinda interesting to look into :) Thank you for sharing such vital information and educating others!

[@silent0089]

Good thing AE3803 wasn't one of those cells but a clumsy sickle red blood cell

[@mariusphilippebulawan4671]

Blood : i got forget my tag name Complement system: u haven't a tag you chose die Blood : owww Complement system : release light Dead blood : nooooo... X _ X

[@MominHashir]

even a lay man can understand the science with this channel. Awesome

[@uchennamaria9291]

This is one of the most beautiful animations/explanation ever. I appreciate you for this. Thank you.

[@JiveDadson]

Can you do one on polycythemia vera?

[@aashishshah6695]

You guys should make more of these videos its does help us as a medical student

[@artiomvas]

Awesome animation

[@ritikatrivedi7468]

Best animation to explain this .

[@davieugenio6495]

i hate how such a amazing video is getting constantly memed by people without sense of humor nor intention to understand

[@soulfulsinger7447]

Such an amazingly done video. Bravo !

[@elizamaria3008]

Brilliant work!!!

[@Kham_Gualnam]

I had to go to the hospital before and I got a lot of “needle” shots

[@fauxvier8519]

Curious!

[@jayvoncampana7231]

Nice and beautiful...

[@ibrahimssen]

Nice video, thanks.

[@carocardozo1507]

Genetic engineering is going to save and change so many life

[@maxjpz]

Interesting to have this recommended, but very informative so I don't mind it.

[@elanimadorcolombiano2547]

why this is demonitice by covid-19 he don´t say the word in the whole video... wtf youtube?

[@muna-cv3wo]

Correct, 👍

[@TheAlextv100]

For anyone who is more curious about this disease and the current leading theory behind the pathology: While we sleep, our breathing slows down, meaning that there is a decrease in oxygen in our blood. Importantly, due to metabolism, the production of CO2 will continue as we sleep. Due to CO2 having acidic effects, we will find a decrease in blood pH. This decrease in pH is what activates the Complement system as we sleep (which is an entirely normal process). In normal patients, our Red Blood Cells carry a few proteins that prevent the Complement System from attaching and attacking. These proteins are called CD55 and CD59. In patients with PNH, they lack the ability to SECURE these proteins to their cell surface. This is because the PIGA gene cannot produce the appropriate anchor proteins. In other words, patient's with PNH fail to create the anchor protein needed to hold the CD55 and CD59 proteins in place on their cell surface. Therefore, without CD55 and CD59, the red blood cell is defenseless against Complement when it is activated in our sleep. When Complement binds to a cell, it will literally punch holes into that cell and cause the cell to "bleed out." Almost like getting hit with a 12 gauge slug to the chest hundreds of times. Hemoglobin is very toxic to our cells, so when it leaks out of the red blood cell, our liver will quickly dispose of it by sending it to our kidneys to be urinated out. This is why patients of PNH wake up with dark red urine when they wake up! Hope this helped further explain the disease.

[@muna-cv3wo]

Correct, 👍

[@itsquran]

thank you for this video its amizing

[@nitinnarwade4075]

Mind blowing. The perfect scientific illustration for the people with non-biological background.👌👌😊😊

[@mrnerd73]

Excellent presentation.... And animations are soo cool❤️❤️❤️❤️

[@jamesizaacjaafar]

Paroxysmal Nocturnal haemogloblnuria or pnh 0:11 0:11 0:11

[@jasonchen9916]

jokes on you i eat 10 vitamin gummys each day

[@JP11155]

Am I the only one who thought the thumbnail looked like Kirby/Kirbies inhaling something?

[@Santy_4799]

Beautifully animated holy shit

[@DaGreyCar5734]

Wow awesome animation.

[@vivekanandabhat]

Extremely good

[@OMA407]

A guy in turban was a researcher... I liked 🎉😂that

[@NPC1173]

No CD55 CD59 complement attacks rbc

[@MrDESHAN93]

Great job. 🤩

[@Snaomib]

the sound effects went crazy in this video lol

[@ОксанаТульпа]

Bit the red bone marrow is most responsible for blood .

[@gregorymalchuk272]

Can they genetically modify the defective hematopoietic stem cells to the correct state, then reinject them as an autograft? Also, what is the typical penetration of this mutation? Does every cell in a victims body have it? If not, the genetic engineering part might not be necessary. Just separate and culture the healthy stem cell lines, nuke their bone marrow, and infuse it back in.

[@Polaris97]

Wow! This animation is very amazing. You get entertained while learning at the same time

[@hennabyrg9053]

Amazing video 😍

[@shahirajgill]

Wow! One of the Research wore Turban.

[@coolboyplay_1257]

Ok

[@chowdhuryrased4770]

Just wow thanks

[@nnjz679]

nice video!!

[@RandomGuy-gw1cu]

watching this for no reason lol🧿

[@kaserl2961]

The title sounds like a meme

[@Mahdi.alkawaz]

Really good work guys

[@AtlasReburdened]

So is that caused by a single letter mutatuion?

[@akn3480]

yes, Unfortunately

[@AtlasReburdened]

@@akn3480 Well, that at least opens it up to being in the first group of conditions that could be outright cured with CRISPR. Doesn't that sound nice? We can eventually eliminate all the single letter mutations and nobody has to do anything _fuckin crazy._

[@akn3480]

This process would be very hard and confusing. Although Crispr/ Cas9 is an awesome work, it's not work as we think. It could be cause of a new mutation or some protector cells could be less effective against viruses. After all those cool things, we're all in the beginning, we'll have see lots of thing after Crispr and i believe, we'll found a cure for PNH mutation. Nobody wants to pee blood. I mean, of course it'd be fucking cool, we can make a real baby yoda!

[@glinda9243]

@@akn3480 my daughter was finally diagnosed with pnh and strangely my twin brother's son has the familial cousin of the disease called spherocytosis. I have autoimmune diseases and have read that there's a 35% chance that your child will also have one.

[@tyty-dg7cx]

Hay

[@Slapper9000]

RIP Blood cell

[@FayeRosewoodMusic]

Maybe some day, specialized blood robots will be able to detect whether a blood cell doesn’t have an authentication stamp and block the inspectors from destroying it.

[@debeshchandrasahoo1061]

z

[@komedi8902]

poggers

[@glinda9243]

Our daughter has this. She recently lost her pregnancy. "Kaitlynn's S Struggle With PNH" onn GoFundMe.

[@ironphilly2366]

how about something that detects it a faulty cell