HLH I Hemophagocytic Lymphohistiocytosis - cause, pathophysiology, investigation and treatment

"HLH is a syndrome of excessive inflammation and tissue destruction due to abnormal immune activation. The abnormal immune activation leads to a hyperinflammatory immune state which is thought to be caused by the absence of normal downregulation of macrophages and lymphocytes.
HLH can be classified as primary or secondary. Primary HLH is hereditary and caused by defects in perforin or granzymes B mediated toxicity. Secondary causes of HLH include infections (EBV), malignancy and autoimmune disease."
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[@veemon9280]

Your ability to break down even the most rare diseases has improved greatly over the years. Now I have a better understanding of one of THE FLASH's villains.

[@akelada]

I just wanted to thank you for providing this content for many years! I have been watching your videos since I was in medical school and throughout residency. I continue to watch them to refresh my understanding. Thank you!

[@shelleycharlesworth5177]

My husband was 74 9+ years ago when he died from HLH. He had been healthy all his life. He got a cold & cough Jan 21 2014. Cold and cough went away in 8 days but he did not regain energy or appetite. I took him to urgent care Feb 4 . Xray showed lungs clear but blood work was off so he was hospitalized and bone marrow biopsy showed Lymphoma AND HLH. That was feb 14. Chemo was begun. He took 4 treatments and developed a lung infection. He died March 27th 2014 after suffering terribly. My world collapsed and will never be the same. We were married 39 years when he died. I had never heard of HLH and doctors told me they see 1 case a year in that hospital and it's mostly in children under age 3. Mostly my husband had good care during his 39 day hospital stay. We were both shocked and terrified. He had been so healthy and we had never heard of this HLH. I will never be the same. He was my rock and I miss him more than ever- 9+ years of being without him sucks.

[@KoreTM]

You explained very beautifully, that i was able to understand without any further details.So much thanks for your great work.

[@hammerradiology1470]

Mix Knowledge, Hard work, Patience and you get your Videos :) I am in awe of your persistence and ambition. Thank you for sharing your knowledge and setting an example.

[@CrankyPantss]

Beautifully explained and diagrammed.

[@Dan-dw3fm]

so so so much helpful!!! Thank you very much for this content!

[@samirsahu2490]

Excellent explanation

[@darinmohammed5119]

Very useful explanation thank you so much

[@kitterzy]

This made much more sense to me than it ever did when I was in college for med tech.

[@Thepersianpopinjay]

I remember hearing the horrifying case of a family who brought their 2 month old baby in to the ER for excessive vomiting after being minorly ill for a few days, everyone expected it to only be a 48hr stay getting started on some antibiotics and some rehydration, but after sudden unexplained rapid organ failure, an HLH dx was made with the infant passing only hours later. What appeared to be a minor infection became lethal in less than 4 days.

[@emmanuellumonga5165]

This is excellent 👍 insight

[@shyamprasad9787]

My mother is in ventilator because of this HLH diesease and she had chickenpox in 4 days it become more critical and now she is fighting for her life

[@appreendixdecostrix9209]

✌ Ok, I left thumbs up! 👍 Nice video. 🎥 thx

[@le_med]

This would be a great title for a band.

[@tsoutsounami]

Death metal band?

[@azzizajane1230]

It’s not uncommon for NK function to be zero. Also, EBV and other viruses can also trigger primary HLH. MAS is very similar but lacks the clear diagnostic criteria that HLH has. When my daughter first had HLH in 2005, we were told there was a 100% death rate within 8 weeks of symptom-onset. When she started to relapse in 2017, it hadn’t reached full diagnostic criteria so she had more treatment options.

[@erinezoller]

A few important immune players to mention. First, that dendritic cells in the tissues and secondary lymphoid organs are also contributing to over activation. Second, while the macs and DCs are pumping out cytokines because they can’t be killed, the CTLs and NK cells (that can’t kill) are continually being stimulated by the macs and DC’s. The Interferon gamma from these over activated NK and CTLs are a HUGE part of the cytokine storm and that IFN gamma is specifically what causes the macs to start eating the blood cells. So while tissue damage by cytokines certainly contributes to organ failure, severe anemia in a matter of days or weeks due to macs eating all the blood cells can drive organ failure as well.

[@serge9259]

You are GREAT

[@pkumar6009]

Thanks a lot 🙏

[@lanoharu4286]

Thanks for your hard work I love your channel and I have a request can you make a lecture about toxoplasmosis please

[@csbrprasad]

Excellent

[@jayakaviyah]

Superb 👍

[@praneet8502]

If u don't mind can you answer me that Which app you use to make videos armando?i mean in which software you are writing like this

[@greysileydiadrianosalazar8944]

muy buenos videos y mas con los dibujos. ( very good videos and more with the drawings)

[@MauriceLeviejr]

Could you make a follow-up on treatments?

[@ok-kb7yz]

I used to like the way when u used to call your name as a intro. But now u directly start with the lecture. 🥺

[@armandohasudungan]

hey thank you haha I thought people liked to go straight into the content ;)

[@CrankyPantss]

@@armandohasudungan Both ways work.

[@questlove7377]

@@armandohasudungan There is a calming effect and enjoyability to hear something that is consistent across all the videos. And yes that does lead to more likability/getting-addicted-to

[@miguel1353]

you draw every good

[@m_m786]

Nice 👍

[@docash6286]

Thankss

[@heenasaifi8506]

Plzz make a video on syphilis

[@_abhishek_here]

Please provide pdf of your lectures🙏🏻

[@Romeo14146]

Sir plz make a video with adrenergic and cholinergic pharmacology

[@andrinnabrown8375]

Can you please do one for ADENOMYOSIS, I've just been diagnosed. Thank you in advance x

[@drvsvr]

Saya pikir kamu orang Indonesia (karena Hasudungan itu nama Batak). Ternyata orang Australia. You did great works!

[@farheensayyad4021]

Please upload a video on pseudosaccharides

[@kathryndevries972]

Hello I am not a Doctor. This was very clear Thankyou. I had a family member diagnosed with CMV with traces of HLH. What does that mean? Age 65 and had allergic reaction to a shot for Neuropathy. Can anyone explain this. Much appreciated.

[@poolejon100]

A friend in his 50s died of HLH a few months ago -the diagnosis was too late. I have concerns around the mRNA vaccines and whether these may trigger HLH in some folk? If so, this could be a very serious (if rare) reaction. I’d appreciate your thoughts…

[@toolcartel4213]

My sister recently passed away from primary HLH. I strongly believe it had something to do with COVID or the COVID vaccine. She was a healthy 42 year old, all started with a fever. I believe something activated the disease. We are heart broken, she had so much fight till the end 😢. She lived about 6 months after being diagnosed. In San Diego it was a rare case and it was hard to treat with so little information on treatment our goal was to get her healthy enough to receive bone marrow transplant, but unfortunately strokes, possibly from their medications made it difficult.

[@jerrimenard3092]

No live virus is used in mRNA vaccines. Perhaps COVID infection could have done it, but not the vaccine.

[@BeenaBasraFoodSecrets]

Nice.

[@bolimeradinesh2638]

Sir ur videos are so usefull for my study 😍 tqs a lot for dat 😍 plzzz do a clip on blood transfusion ...plz🙏🏼 a student request from india 🖤

[@panda-pk7zi]

Sir can you please upload a video on skeletal muscle relaxants. Please🙏

[@mohonhlanghu3465]

So much studies being done and couldn't come up with whats the primary cause and its cure

[@stevekay5486]

My son died of this in april 2021 aged 28. He had had an ear infection for 2 weeks before he was admitted to hospital. He died 3 weeks later. What a horrible disease it is. He never had an ailment in his life up to this.

[@Horseyloverxoxo]

I am sorry for your loss. If you don’t mind can I ask the symptoms your son experienced? My dad is being tested for it, he has had lymphoma treatment chemo and is bed ridden with extreme fatigue and confusion/memory loss.

[@stevekay5486]

@@Horseyloverxoxo He had enlarged liver, enlarged spleen, heart problem, they said his brain was being affected too. He had very low blood counts and fluid on his lungs It killed his liver which he needed to fight infection

[@Horseyloverxoxo]

@@stevekay5486 thank you for taking the time to reply.

[@skankhunt4220]

The exact same thing happened to my best friend this month his condition became critical within a week he died a few days later what a horrible disease

[@stevekay5486]

@@skankhunt4220 You are right, a bloody horrible disease. I wouldn't wish it on anyone

[@ksajsmuszkksd9126]

Ty

[@staystrong9238]

Plz upload topic related to microbiology, pathology & bio chemistry plzzzzz

[@sudeshnadasgupta119]

Can it occur suddenly after any nominal infection??

[@neilbutters1911]

Lost my dad to this last week

[@heenasaifi8506]

Plzz explain about syphilis it's treatment and control, all about syphilis plz plz itz a request

[@sayaneha08]

What is the treatment. Chances to live.

[@millipedemiranda1339]

You should have more subs

[@hammerradiology1470]

I think so too! I really appreciate people sharing their knowledge...

[@sairam8140]

Hii bro i need physiology of digestive system

[@chloenichollea.lincopines3215]

Hi, I'm a Nursing student, our Midterm exams are about these Case Studies and I picked a case study about a Patient having a Hectic fever, and I didn't understand what Hemophagocytic Lymphohistiocytosis Now I can understand more what it is and why it caused such a Hectic fever on the patient. Thank you so much! ♥️

[@vaishnavikarkada6954]

video request on antivirals pleeeeease :) You're the best

[@greysileydiadrianosalazar8944]

Hello . Please, the videos must be subtitled in Spanish, I'm from Peru

[@MadhubhartiGBava]

Thanks lot Sir, would you like to make on fate map.🙄

[@cargumdeu]

pretty much described the effects of covid there...would zinc, calcefiodol, prevent cytokine storm?

[@kanikathakur3835]

sir pls upload a video on the topic implantation in human reproduction

[@user-rr4zs7il6t]

المبلغ اللي ابي معي

[@lydiahelmick8092]

A recent death of fully vaccinated sixty year old woman of this very thing brought me to your channel.

[@amirhosseinsharifi19]

👌👌👌👌

[@jph1503]

❤

[@misterB2231]

The causes need to get enlightening, you did't talk about lymphoma lupus and still's disease.

[@nightstarking]

Can the covid shot increase your risk for this type of cancer

[@muhammadmohsin8584]

I lost my brother on 26th January 2022 due to HLH, who was only 25 years old and was so handsome 😥😥

[@stevekay5486]

my son too april last year, my condolences to you.

[@muhammadmohsin8584]

@@stevekay5486 how old was he

[@muhammadmohsin8584]

@@stevekay5486 My condolences to you too

[@stevekay5486]

@@muhammadmohsin8584 he was 28

[@muhammadmohsin8584]

@@stevekay5486 I'm so sorry, he was too young, any idea how he got caught to HLH

[@proffezur]

Yeah... Just name it after someone and call it a day. That's way too much of a mouthful 😅

[@EnglishMadeEasyWithSri]

My sister's husband collapsed due to this hlh...Is there a chance that it spreads to her???? someone please reply...we are so affected by his death...

[@erinezoller]

It is not contagious.

[@user-rr4zs7il6t]

ابي سفه .٥٠.ريال.بسام

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Good intention is happiness and it is a solution to every problem I will stick to the good intention, even if it is the cause of my death, but my beloved is God knows more than me that good intention is happiness and it is a solution to every problem. If the person with good intention is sure that God will help him, God will help him. Here are human demons and jinn demons either admit their mistakes or deny The truth is that their fate will be hellfire, and their fate will be miserable The characteristics of human demons and jinn demons are arrogance, selfishness, sarcasm, bullying, and other bad qualities that harm society. I love the fire of hell because it will torment everyone who does not respect God's tests I love telling the truth about all the endless sciences, which is deliverance from God’s torment My profession is to do good for nothing in return for sacrifice, not for interests When will those who do good deeds for free by sacrificing hold high positions so that reality becomes more beautiful and in the utmost justice and equality between the strong and the weak???

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