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Blood Physiology Lecture

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Summary

Join Nicole in her engaging session exploring various aspects of hematology, often considered a daunting subject by many medical professionals. Nicole embarks on a deep dive into important concepts such as blood cell development, hemoglobin, clotting cascade, and associated disorders. She also plans to brief you about some widely known malignancies like leukemia, lymphoma, and myeloma. From the physiology of blood formation to clotting factors and common blood anomalies like Sickle cell anemia and various forms of hemophilia, Nicole seeks to deliver this session with comprehensive clarity. Be prepared for this on-demand learning experience, which includes a practical guide to reading and understanding coagulation screening tests, essential in your daily practice. Young and senior doctors, don't miss this chance to refresh, update, and enhance your hematology knowledge!
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Learning objectives

1. Understand the basic physiology and cytology related to hematology, including the formation and function of blood cells. 2. Gain knowledge about common hematological malignancies such as leukemia, lymphoma, and myeloma. 3. Learn about various blood disorders such as sickle cell anemia and hemophilia, and understand their impacts on the body. 4. Understand the process of hemostasis, including the role of platelets, coagulation factors, and the clotting cascade. 5. Being able to interpret results of coagulation tests, such as prothrombin time and activated partial thromboplastin time, and understand their implications in diagnosing disorders.
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Computer generated transcript

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The following transcript was generated automatically from the content and has not been checked or corrected manually.

Hi, everyone. Um I'm Nicole. I'm just one of the, it's just gonna be taking um a wee session today, so probably leave it until maybe about five past just to um give people a chance to um if anyone else has joined in. Uh and then we'll make a, we start. So thanks very much. I'm just double checking. Can you see my slides? Everything? Ok. And they're like moving all that kind of zoom stuff, right? Sure will make me start the are all right because I'm sure he's all wanna get away cause it up. So, thanks um very much. Um everyone for coming. Um So today I'm just kind of talking about some of the main stuff that you kinda need to know for hematology. Um I know it's kind of everyone's like probably least favorite subject um and be with me, bear with me as well cause I'm in the, on the internet and group. Um but um we'll get through it here. Um So just in terms of what we'll cover, so I'll just to start off, I just kind of go over like just a wee bit of this quick vision um of like some of the physiology that you need to know. Uh And then I'll kind of cover um, some of the main malignancies as well as you need to know. So like just very, very quickly briefly going over, um, leukemia lymphoma, myeloma cause I feel like they're kind of covered pretty well well, by next year, finals and pass and everything. Uh, and the Queen's lectures as well, I think, um, mainly cover like the malignancies as well. Uh And it'll just kind of cover some stuff as well that I feel like it wasn't really covered um whenever we were in third year and you kind of have to learn yourself. Um So just going over um like things like cycle cell anemia and then some of like hemophilia like Mill's disease um cause hemophilia actually come up in one of our kind of a a case and I think everyone was um through quite a bit back because it was really um covered. Um all that much. Um So kind of just starting off with um good over a bit of um physiology um which is everyone's favorite subject, I'm sure um on the third. Um So I'm sure everyone knows um in terms of what your blood is actually made up of, um it's mostly plasma and that's like where your, all your electrolytes and like your clotting factors, proteins, um they're all dissolved in your plasma. Uh And then it's the rest of it is just made up of your red red cells and then your white cells. Uh So, in terms of where blood cells um actually come from. Um So they all developed in the bone marrow. The bone marrow is kind of like the blood making factory um uh from these kind of hematolytic stem cells. Um So those stem cells kind of um either go down one of two lines. Um So they can become myeloid cells uh or lymphoid cells. You don't really need to know like all the different stats, just kind of know. Um you just kinda need to know just the basic terminology. Um because then that will kind of just help you understand what cells are affected. Uh whenever you're learning about leukemias. Uh so your myeloid uh cells uh or like your red blood cells and then your myocytes and then your platelets bought off from the mega pericytes. Uh So your platelets are myeloid cells as well. Uh And then your Golos uh are myeloid cells um which is working. Can I get a bit confusing because some white cells are myeloid cells whereas, whereas the white cells are lymphoid cells. Um but your neutrophils, your ails and your basophils uh on your monocytes are all myeloid cells as well. So they'll be affected um and abnormal and myeloid leukemias as well. Uh And then, and uh your medical proliferative disorders as well. Uh Are your polycythemia rubra, vera or your red blood cells are really, really high, uh, on your, uh, essential thrombocythemia as well where your platelets are really, really high. Um, you might see those can imagine they're covered quite a bit on polymed as well. Um, and I think we were ta yes on them in third year, in the third year or final year. I can't remember. Uh, but, um, that's just kind of something, uh, I'll not cover them here. Um, but they're kind of easy enough to go over. Um possibly kind of covers pretty much everything you need to know. So I'm not um teach you to OK, on this. Um So in terms of them cells that come from your lymphoid lineage. Um so that's basically just your lymphocytes. So if um if you're gonna remember everything, just remember that your lymphoid cells or your lymphocytes and everything else is your myeloid cells. Um So that just should be lymphocytes, your two lymphocytes and your no color cells and those are the cells that'll be on abnormal then in lymphomas and in your um lymphoid leukemias, like your lymphocytic and your lymphoblastic leukemias as well. Um So moving on then just the hemoglobin. So just very, very quickly, briefly going over it. So we all know um it's a protein that carries blood around your body, delivers oxygen or carries oxygen around your body, even delivers oxygen to all your tissues. So, um normal adult hemoglobin is made up of your two alpha. Um change or to be a change. Um But you have kind of variants as well. So you have your fetal hemoglobin, um which kind of stops being produced a few months in life. Uh And it's predominantly produced in the fetus as the name suggests. Uh And then your uh H pa too as well, which you don't really need to kind of pay much attention to that, but that's made up of two alpha globin units uh and two units. Um I just kind of included it just because it was in this diagram, you don't really need to know what it is. Um But it can be raised in people who have beta thalassemia. So just in case you come across that whenever you're reading and you're, you know, wondering what it is, but I wouldn't kind of spend too much time worrying about that. Um So briefly as well. I know um clotting and like clotting cascade is something that I still kind of struggle with on the hip that you mentioned to be honest. Um But I just thought to cover it just kind of um because it will be covering clotting disorders uh later on and then kind of talk a little bit about the coag screen as well. Um And that's so hemostasis. Um It's just a fancy name for um blood clotting uh and issues with it is what leads to, you know, your abnormal clotting or your bleeding disorders. Um So the kind of initial step of um of hemostasis is your vasospasm. So, if a vessel becomes damaged, uh the kind of smooth muscle cells in the walls of the vessel will kind of react to that uh and sense stop and then they'll just contract, they're just trying to restrict that blood loss and kind of minimize the area of damage. Um Usually that's probably not really enough to stop, uh you know, a big bleed. Um So then whenever the vessel wall is damaged, that will kind of expose collagen uh to kind of everything that circulating in the blood. Uh And as the platelets kind of being carried along in the blood and there uh they come into contact with without exposed collagen, they'll stick to the collagen and then they'll kinda stick to themselves. Um They'll track more platelets that'll stick to the collagen, stick to the other platelets uh and kind of just go around and around in that cycle, just attract more and more platelets um to the area uh to kind of form the platelet plug uh to kind of help uh better uh stop the blood loss as well. Uh So, platelet adhesion and that kind of cycle of the platelets become sticky and kind of stick to each other uh is helped by the milligram factor. Um So sure is probably all hard by now. Um A VV Brown disease uh and that um is kind of associated with um like mucosal bleeding um which can also be seen in uh when you have low platelet levels. Uh And that's basically just because Bone Miller Brown factor kind of um helps the function. So with like uh with level was 1 mg factor, um you're gonna have similar um kind of symptoms as if you have low platelets. Um So you're gonna get um those kind of symptoms of like nosebleeds, um like uh heavy periods or uh like that kind of thing. Uh So, moving on the end of the cascade, I'm not, we wanna talk about this um in detail because you really don't need to know it. Um But it's basically just because platelet plugs aren't um really the most secure thing. Um So you're gonna need something else to kind of strengthen that uh and make an actual clot. Um So that's where the clotting cascade kind of comes in and that makes your fight liver mass not stabilizes the platelet ga mix, get a proper clot uh that is going um hopefully stop uh bleeding completely. Um So all you really need to know what's true pathways. Um the intrinsic pathway and the extrinsic pathway and they kind of come together um uh to, to kind of activate uh the common pathway that thing goes on. Um They've actually formed your fibrin and then your stable cloth. Uh The intrinsic pathway is kind of activated by um similar to the platelet medication. Um It's kind of activated when clotting factors come into contact with an abnormal surface, which is usually they, um, they like, expose collagen whenever vessels damaged. Uh And that's why that's uh kind of, uh that's why that is uh initiated. Uh The extrinsic pathway is kind of um, just activated by tissue damage, I'd say to the actual vessel itself. Uh So that's all I'm really gonna kind of chat about. Um, you really don't need to, you know, sit down and learn it. I'm probably sure you, I'm sure you all probably know that yourselves. Um The main thing, it just kind of helps with um understanding different parts of the coag screen. Um So I'm sure you are probably all seen a co wire in my eye as well. Um So the main kind of things that are called wide screen will show you uh are the prothrombin time. Um And they activate a partial thromboplastin time, which is kind of hard to say, but of my foot. Um But basically, uh the PT or the prothrombin pain just basically measures it kind of reflects the extremity pathway. Uh And the only factor that's really involved in that pathway if you go back to the diagram, um that it kind of showed before is factor seven. and factor seven deficiencies um are actually quite rare. Um So usually if you see a prolonged prothrombin time, um it's probably gonna be something else other than the clot factor deficiency that's kinda causing that prolonged prothrombin time. Um And it's usually stuff like liver disease, like um that or kind of like a deficiency in Vitamin K that is going to prolong your prothrombin time rather than something like a hemophilia. Um So if you're gonna kind of remember anything um about a coag screen, just remember that if you see um the A PTT and it's prolonged and only the A P PTT is prolonged, um it reflects the intrinsic pathway and the intrinsic pathway kind of um involved a lot more factors unspecifical on the, the ones that you probably wanna remember. Side effects factor eight, factor nine and factor 11. Um And pretty much just focus on factor eight and factor um factor nine. because that uh so deficiencies and factor eight is hemophilia a deficiencies in factor nine is hemophilia B and then I was just kind of just for completeness, I'll talk a wee bit more about them at the end. Um Factor 11 deficiencies is not OK to cause your hemophilia C uh which is um just another kind of he uh form of hemophilia, hemophilia that just kind of chatted about just in case uh on the off chance it comes up. Um So if your A PTT is prolonged, um it's more likely to be due, it's more likely to be due to an issue with the clotting factors rather than something else. So, included this way, I kind of find this quite helpful when I was going over things for um for a um in terms of the coag screen. So, again, if you've just got um a prolonged uh prothrombin time, um it's probably um gonna be something uh kind of outside of the actual cascade that's kind of affecting that. So something like um you know, like liver disease, um Warfarin can also prolong it as well if both of them are prolonged. Um It's probably something uh again, like liver disease uh or anticoagulant that on. But again, um if it's just the A PTT that's prolonged you on, then go on to do um the measure bleeding time, it's not really measured um in practice anymore. Um because you have to like make the patient bleed uh in front of you and then measure how long it takes for them to stop bleeding. That's a measure of platelet function. Um So, platelets are fine in hemophilia. So, if the bleeding time is normal, then um they have an increased um ABT uh and everything else in the coag screen is normal, then they have a hemophilia. Uh if their bleeding time is increased, then they have an issue with their platelets and they probably have bone brown disease. Um So we'll kind of talk about uh more about that, but it's just kind of included this cause I think it's a good reference. Uh And then just is gonna say as well. So, Antiphospholipid Syndrome um is included there and it can cause it is, it's a, it's a clotting disorder, um where you're more prone to things like dvts and clots. Um The typical kind of exam question is, um, like a lady that's come in with recurrent miscarriage. Um, she's had, she also had a history of a couple of DVTs or P ES and there's a history of clotting in the family as well. And, uh that essentially just basically, um so antiphospholipid syndrome um kind of paradoxically um prolong your A PTT. And that's kind of um just because phospholipids are actually used, you don't really need to know this, but phos it's just in case it comes up, um phospholipids are used whenever they're kind of trying to work out the A PTT. And if you have anti phospholipid antibodies, um that's gonna get kind of interact with the test. Um But I've kind of included like a couple of wee notes about that um as well, but you don't probably, you don't really need to know that just in case you were wondering why, um why that kind of prolongs your A PTT and it's not an actual bleeding disorder. Um So moving on then, so we'll forget all that and then just um move on just quickly to talk about some of the main malignancies. So, in terms of leukemia. So, leukemias, I'm sure you all know um are cancers of your right blood cells. Um And they're kind of what we call liquid tumors. So you might hear people talk about liquid and solid tumors. So, um solid tumors are, you know, um tumors that are, you know, fixed in one place and then kind of spread to other places slowly. Um Leukemias are liquid, they're in the blood. Um We don't really use no normal staging kind of methods like, you know, like your chicks criteria, that kind of thing. Um or like stage one is like confined to the tissue and like stage four is like, you know, uh like distant mats or whatever. Um because because they're in the blood, they're just all around the body all at the same time. So that kind of staging doesn't really um moder. Um and you don't really need to know what any of the subtypes and in detail are all kind of like genetic um test and to like work it out, that's like way beyond what you would ever need to know um for medical school. Um So just um you might just see them as well just being described as um monoclonal and that basically just means that it was one cell that kind of developed a mutation uh and then just cloned itself and grew and grew and grew um to kind of form the leukemia. And whenever you look at a sample of leukemia uh of leukemia cells, they'll all be genetically or they generally are all um genetically done uh identical because they've all derived from that one cell. So they're monoclonal. Uh So, in terms of classifying the leukemias, um basically, all you need to know is that um they can either be acute or chronic, um which is basically how fast leukemia is progressing. Um So your acute um leukemias tend to be come on quite a lot, um a lot quicker and uh they're associated with a high number of blast cells which is just immature cells. Um uh in terms of um so they're being produced so quickly. Um And the leukemia is progressing so fast that the these immature sales are just being released from the bone marrow into the um into the bloodstream um without kind of uh having a chance to really grow mature. Whereas chronic ones um are a bit more uh slower progressing and uh the sales actually have time to kind of mature or the uh proper lymphocytes, even though they are still abnormal. Um, blast still can be uh present in chronic leukemias as well. Um kinda trip people up. I know. Um So CNL came up a couple of years ago and I think people were a wee bit confused because they were given like a blast kind. Um And a lot of people kind of mix that up with an acute leukemia, but it's all about uh if there are mature sales present there, it's um more than likely gonna be a chronic leukemia. If it's mostly blasts, it's gonna be an acute leukemia. Um So then the other way that they're classified as well is basically um whether lymphoid cells are affected or myeloid cells are affected. Um So that's kind of what I was talking uh about earlier on. Uh in terms of like whether basically your lymphocytes are affective or are affected or whether it's like your granulocytes and your uh megakaryocytes um that are affected. Um and that's just basically heart you subtype, but you don't really need to know anything more than that. Um So in terms of how leukemia presents, so because the leukemias generally are growing in the bone marrow as they grow and grow, um they'll take over the bone marrow and they'll cause bone marrow suppression. Uh And that, ok, and they cause your pancytopenias and then you'll get the um uh you'll get symptoms associated with low levels of your red cells. So that'll be your anemia, symptoms, like your fatigue or short breath. Um You'll uh look a bit pale, you have like polar, that kind of thing. Uh As your white cells uh are the white cells that are being produced are abnormal. Uh And uh so you'll be more susceptible to the infection then uh because um the uh uh there's not as many normal cells being made. Uh And then your platelets will be uh uh your platelet production will decrease as well and that'll lead to, you'll be more prone to bleeding, easy bruising that kind of thing. Um You can have other symptoms as well, so you can get bone pain. So these cells are growing in the bone marrow. If they kind of um in into the bone itself, you can get bone pain. Um So because the bone marrow kind of isn't working as effective as it is. And you're having less pancytopenia, you're not having kind of um high enough levels of normal c normal blood cells. Um you can uh start to form blood cells uh in other places that you normally don't. Uh And typically this is in your liver and your spleen. Um So you can get apono megaly as well um as a presentation of leukemia. Uh and that can be associated with abdominal pain as well. Uh Because those two organs just get so big and kind of start to press on other structures and that kind of thing. Um like every other cancer as well. It's um associated with fever um of unknown origin as well. And then if uh the cells spread into the lymph nodes, you can get lymphoma as well. Uh And you can also um it's a bit confusing but you can either have increased or decreased white cell counts as well. And that kind of just um uh depends on uh how abnormal cells are and kind of like how they're measured. Uh But just keep in mind that um you might see um and you might see it coming up in a exam question that the white cell counts are increased or decreased. But you can kind of look at the, um, the whole picture to figure out that it is actually leukemia even though, um, they might have uh a lower than expected, um, white cell kind. Um But generally, I, if you're gonna be tested on this in like an or an oy, you'll probably more than likely have an increased white cell count just to avoid um kind of an confusion. Um So, moving on into the actual cell types, um so a ll um kind of is originating from your lymphocyte um precursors. Uh And this is uh like a cancer that's mostly consistent of lymphoblasts, which is just IMA uh lymphocytes. Um It's the most common uh malignancy that you can get in childhood. Uh It's more common in boys and it's associated with Down Syndrome as well and uh as well as presenting with all the other kind of sys in terms of leukemia that I was mentioning earlier on. Um Sometimes they might talk about um it can present with testicular swelling as well. Uh And that's pretty much all you need to know about A LL um it's covered a wee bit more impedes as well uh rather than just um hematology for third year as well. Um So, in terms of your other acute leukemia A ML, um it's uh is going to originate from your myeloid cells. So it's mostly myeloblasts and uh the myeloproliferative disorders that I was chatting about earlier on and they can transform into a ML. Uh So, in terms of what you might say or what they might describe uh in terms of an OS or an M CQ, um our rods are kind of like a classical of this and these are the kind of purple things that the, ours want to. Uh and the CS are just kind of like Granules of something um that just stand up. Uh And they are pretty classical um of A ML sales. Um So there is a, there's a subtype of a ML that is tested fairly regularly on pod. Um So it could come up. So I just kind of child about it. A ML can be quite serious, but this subtype of, of uh a male, those were um is also kind of even more serious and just kind of normal um a male. Um So you might have heard of it. Um It's acute promyelocytic leukemia. It, I just remember it came up all the time um whenever I was going through post me. Um so patients with uh APL or acute myocytic leukemia can actually present with um D IC. Um So they'll present really, really ill um when you do um like a coag or like a full blood count, they'll have um decreased platelets, decreased fibroma. Um And uh just kind of the range coagulation um and included a kind of like a wee bit more, sorry, a wee bit more information on not just the notes, um cause there's like a translocation that's um related to uh APL um that they kind of test sometimes as well. I haven't talked about any of the genetics in this really because I think there's probably nothing worse than listening to me. Um, list off a load of numbers and um genes. So, um pos has a really good summary table on it. Um It's just one of those things that you just have to learn, unfortunately. Um um, but that's probably one of the most common ones that is tested. Um So then they know the CLL. Um So CLL is really commonly tested as well. Um So it's um your chronic leukemia. So, um most of the cells here are gonna be um abnormal mature uh lymphoid cells. Um So the classic picture will be um like an older adult who's um come in asymptomatic. Um They've had a full blood count for some other reason and they've picked up this really increased white cell count. Uh And when they uh go to do a blood film, um there's these abnormal cells and uh you'll also find smudge or smear cell as well uh on the blood film, which is basically just these cells here in the middle and I hope you can see my cur they're pointing at it, um which is basically just because those abnormal cells are so fragile uh that they just break apart uh whenever the blood film is actually being made. Um So they could very easily show you, you know, in and all or in an M CQ, give you someone with like a really raised white cell count and then say that um there's also smudge cells or smear cells uh on their blood film and then, um it'll probably, it'll definitely be CLL uh so CLL, even though it's generally asymptomatic, um if someone suddenly becomes symptomatic and they have, they developed lymphadenopathy or they're getting drenched and night sweats or any of those b symptoms, you're gonna uh worry that um the CLL has been um actually transformed and uh uh high grade lymphoma. So that's called your Richter's transformation. Uh And it usually kind of transforms and a diffuse large B cell lymphoma, um which is quite like a uh it's quite a serious uh a serious um and high grade lymphoma. Um So just keep an eye out for that as well uh in questions or in case as well, um that you always wanna be really like um that it's not something more serious. Uh Generally CLL, you can just let um you can just kind of watch and wait uh and just keep an eye out for those symptoms. Um uh But generally it doesn't really need any treatment uh and then moving on to CML. Um So it's gonna be um composed of your mature myeloid cells uh that are gonna be abnormal and this is also tested all the time as well. I think we were tested on the third and final year. Um So it's associated with the Philadelphia chromosome, which is your or um t 922 translocation. So, translocation between chromosome nine and chromosome 22. Uh and that results in the um this B cr able fusion protein um which ters kinase inhibitors can um directly target. So that came up in our finals this year um where they uh where they said that this person had um a CML with a Philadelphia chromosome, what class of drugs um was used to treat it? Uh And it was a tyrosine kinase inhibitor, um which your, your first line is usually uh imatinib and that's probably the main um thing that you need to know. Uh It's one of the main kind of chemotherapies as well. That's um that's tested um even um kind of across oncology as well including hematology. Uh And then you can measure whenever someone's on imatinib or on a tyrosine kinase inhibitor. Um You can actually measure their levels of BC are able um just to make sure that they're kind of um having a good response and they're maintaining in remission as well. Um So that could be, you know, um just uh um that could come up as an M CQ or they could ask you that in the as well. So, that's just something. And keep in mind. Uh So, moving on them from leukemia is just on the lymphoma. So, really quickly. So they originate from lymphocytes, uh and lymph nodes that are rather than being liquid tumor, uh like leukemias, they are solid tumors. Um So your typical symptoms are probably usually gonna be your painless lymphadenopathy and then your b symptoms as well. Um which is um your fever, weight loss, join and night sweats, that kind of thing. So there's loads of different sub types of lymphoma and I haven't covered them here because again, there's probably nothing worse than sitting someone just listening off a load of different things. Um So basically, probably the main kind of thing that you need to know is whether or not it's a hodgkins or non hodgkins lymphoma. Um So in Hodgkin's lymphoma, they're kind of are, they're um characterized by these re starg sales, which is the, um they might also be called, like you might see them being called nuclei or cells as well because apparently they look like ows to some people. Um So if the red starg cells are present, it's a Hodgkin's lymphoma. Um And also another thing that you might see in MC QS, it doesn't really happen in real life, but MT Qs are M CQ land. Um So if someone, one has pain in their lymph nodes, um that's triggered whenever they drink alcohol like after a night um of uh of drinking alcohol then, um, that suggests a Hodgkins lymphoma as well. So Red Starbucks sales aren't present then in non-hodgkin lymphoma and there's loads and loads of different subtypes again. Um, pass med, um, has a good table, I think going through all the main different types. But, um, I wouldn't kind of spend an awful lot of time on it. Um, it's probably, you know, it's not really that high yield. Um, and I've just kind of included this way um table. Um just so you can go over in your own time. It just kind of has some of the main differences between um hodgkins and non Hodgkins lymphoma then um so then quickly just going over myeloma as well. So that originates from your plasma cells. Um So your plasma cells are the cells that can make all your antibodies. Um So they're abnormal. So they're gonna produce abnormal antibodies and that's called P proteins and power proteins can kind of um cause a lot of different problems around the body. So um they can get stuck. Um and um your renal tubules and can cause um your renal function to decrease. Um they don't function normally as normal antibodies. So you, they're gonna um you know, um be more prone to infection. Uh People with myeloma as well are um quite prone to um people with cancer in general, but um myeloma uh especially as well, are also um quite prone to like DVTs clots that kind of thing. So that's something to keep in mind as well. Um So in terms of um I'm sure you all know crab by now. Um So the B and the I were added on whenever I was studying for finals. So, um myeloma, your acronym is gonna be calcium. So they're gonna present with high calcium levels. Um They're gonna present with um decreased renal function, anemia, um back or bone pain uh because the kind of abnormal cells are again infiltrating bone um can cause in your characteristic lytic lesions. Uh and then they actually be in the eye. Um So, because the plasma cells are kind of taken over the bone marrow, again, you can have bone marrow suppression uh and you might be more prone to bleeding because you don't have as many platelets as you should. Uh in terms of infraction again, um as I mentioned, um you're not gonna be able to fight off infection as well as you usually would be able to. Um because you're producing these kind of abnormal antibodies. Um So, um if you're gonna remember anything, remember cro but just kind of keep in mind as well. Um just you're um more prone to infection and then more kind of prone to um bleeding uh as well. So, moving on in um from the um legacies um on to just the hemoglobin of these really quickly. Um So, hemoglobin off these um there are just inherited disorders kind of affecting your hemoglobin structure. Um So you're either gonna have your thalassemias or your hemoglobin variants. Um So in terms of starting off thalassemia, so normal hemoglobin, I kind of chatted about, about it earlier on. So, well, it's gonna consist of your two alphaglobin chains and your two beta globin chains. Um can go to that saying if you have a defect in your alpha globin chain, it's you're gonna have alpha thalassemia. If you have a defect in your beta globin chain, then you're gonna have beta thalassemia. Um They're both autosomal recessive and then they're usually associated um with a microcytic um hypochromic anemia, uh which the cells are small. Um They tend to be even smaller than you would say um in something like iron deficiency anemia and as well. Uh the anemia is just chronic and uh can often be asymptomatic kind of dependent on uh what type of thalassemia you have. So, starting off with alpha thalassemia, this is kind of a bit um It's quite complex to kind of wrap your head around. I still kind of stroll under understand that as well. Um So alpha thalassemia is a bit more difficult to understand because there's four genes that are codon for your alpha globin um chains. So I kind of try to simplify it as much as possible. Um And hopefully, that's pretty much all you need to know. So if you have one kind of alpha globin chain that's affected, um, you're gonna be asymptomatic and you're just gonna be a carrier, um, of thalassemia if you have to, um, globin chains affected, um, generally, again, you'll be asymptomatic but you might just have that kind of chronic anemia if there's three chains affected, um, you're gonna have a more serious disease. So you're gonna have quite, um, severe anemia required, um, a lot of blood transfusions throughout your life. Uh, and then you might also have spina megaly as well. Uh And that's kind of to do with that extramedullary uh hematopoiesis. Uh And then if all four alpha globin chains are affected, that's actually incompatible with life. Uh because uh so pretty much every hemoglobin kind of molecule uh including uh fetal hemoglobin, you need two alpha globin chains for that, at least. Um So if all of the um chains are factor that you're not, you're just not gonna be able to kind of make um any kind of effect of hemoglobin. And uh it's kind of covered a wee bit more impede. So, as well, um it's usually associated with death in utero or very shortly after birth. Uh And that's a kind of a condition called hydrops, fetalis. Um Just in case you come across that uh whenever you're revising for your progress tests. So, in terms of beta thalassemia, it to be a bit more um easier to understand. So either um you've got uh one beta globin chain affected and you've got a beta thalassemia trait and you're just asymptomatic and you have like am chronic anemia that probably won't really bother you much. Uh But if you have two globin chains affected, then you have beta thalassemia major. Uh and that's going to present kind of shortly after, um shortly after birth as fetal hemoglobin kind of stops being made. Uh and hemoglobin kind of takes over. Um, so kids usually present with failure to thrive and that's really severe anemia because they're not making um kind of normal um auto hemoglobin. And uh uh again, it open up the spinal megaly and bone deformities. So usually something called frontal b um where the kind of forehead comes kind of like rounded and quite like large. Um because because to kind of cope with the anemia, um blood is starting to be made in parts of body where it usually shouldn't be. So it's usually always made in your long bones, like in your legs and your arms. Uh But uh it can start to be made in the skull uh in the liver and the spleen. Uh and that's where that kind of uh the bone deformities in spinal megaly come from. Uh and then again, um similar to uh before um people with uh beta in your major will be uh dependent on transfusions uh to kind of cope with the um with the symptoms of the anemia. Uh And therefore, because they're getting uh kind of uh transfusions all the time are actually at risk of iron overload. Uh So you might need to give them um kind of iron agents as well um along with their transfusions. So, uh I just concluded that B diagram again cause I feel like it just kind of helps you understand a little bit. Um more about why alpha thalassemia is kind of so much worse basically because all of these kinds of hemoglobin are at the bottom, all need um alpha um uh Albin in them. Uh whereas um people with uh beta thalassemia can kind of cope a wee bit more um because of fetal hemoglobin as well. I've included kind of a wee bit more um notes on that kind of just in the uh in the notes underneath these legs and then I'll um I'll get them sent out um just after this. So then moving on then um just a sickle cell disease. Um So this is kind of like a spectrum of disease um caused by um just a point mutation. That's kind of uh it's quite um commonly examined as well. So, um it's like a glutamate, um amino acid um substituted by feeling um I mean, what uh it's also autosomal recessive as well. Um So the cycle cell mutation um will kind of result in this um abnormal hemoglobin um called H PS rather than kind of normal hemoglobin. Uh And then whenever that's deoxygenated, um so normal hemoglobin can cope normally, uh with uh with the oxygen kind of being offloaded into the tissues, um, or as the hemoglobin and sickle cell disease, um, whenever oxygen is all loaded under the tissues, um, it kind of, um, sticks together polymerizes and that deforms the red blood cells into these kind of sickle cells rather than being just normal by concave discs. Uh And those sickle cells are a lot more easily destroyed. Um, they tend to stick together um kind of in and block uh capillaries and cause infarction as well. Uh And they're um because they're more easily destroyed as well, that leads to anemia, but it also can lead to jaundice as well. Um So, uh essentially, if you have um sickle cell anemia, you kind of get this chronic hemolytic anemia because your cells are constantly being broken down. And uh the, you know, the breakdown products are formed in bilirubin um giving you jaundice. Um So if you um have kind of one trait or one factor gene, you're heterozygous, um you're described as having sickle cell trait and you're usually asymptomatic. Um if you have uh two factor genes in your homozygous, uh and you have sickle cell anemia and that's a chronic hemolytic anemia that I was talking about. So, um because the sales uh or because of the cycle sales um aren't kind of as flexible as a biconcave disc, they get stuck in the capillaries. Uh and then this can lead to uh what we call sickle cell crises. Um So if it gets blo if capillaries get blocked, um this can, can cause uh like really um severe pain in the hands and feet and swelling in the hands and feet. Uh And it can also affect other parts of the body as well. Um But that's kind of one of classically um be described as in like an AMC Q. Um um And that's basically just because um uh the capella or the blood flow is kind of being blocked, uh like the distal parts of like your toes or your uh your fingers uh and causing that pain. Uh And it can also be associated with fever as well. Um So the cycle, so cells can then block uh the blood vessels in there of the spleen. Uh And that's actually quite serious because the spleen is, you know, like a really big reservoir of blood and blood cells. Um So that can kind of cause severe anemia and hyper hypovolemic shock. Uh And that's an emergency um that uh may uh someone may need how um may need to undergo a splenectomy. Um I've kind of covered as well, just kind of more of the management than the notes under this as well. Uh But it's generally, um they're all generally managed kind of conservatively uh unless they're kind of an emergency like splenic um crisis uh or QT syndrome as well as an emergency. So, aplastic crisis. Um can occur whenever um people with sickle cell disease or kind of any other um uh disease that kind of impacts on the red blood cells um are infected by uh Parvovirus B 19, which is your cheek syndrome. Um So that can lead to bone marrow failure. Um So generally whenever um no or whenever someone with, without sickle cell um disease is that by Parvovirus B 19 that actually causes kind of a, a po of um blood cells which um normally um you're able to cope with, you don't really notice any symptoms of that, but in someone with sickle cell disease or, you know, another um kind of summer condition, um uh because they can turn her blood cells are so high, they're not able to cope with that um with that kind of uh with a pause and the production of new cells. Uh so they go into bone marrow failure or they aplastic crisis. And that's as um characterized by your uh sudden kind of really drastic fall in hemoglobin on uh reticulocytes, which are kind of like your new red blood cells that are being produced. Um They'll also be reduced as well. Uh And then acute chest syndrome as well. Um kind of uh happens when, however, the sickle cells block the vessels in the lung. Uh and that's kind of uh just associated with respiratory symptoms. Uh essentially. So like cough, shortness of breath fever as well. Uh And then uh new infiltrate on uh chest x ray that kind of looks a wee bit, um kind of just like patchy kind of changes, uh do a chest x-ray. And uh so that's basically um all you need to know about the crises. It comes up on pa or on past men and quis men quite a lot. Um So then moving on, then just the clotting disorders. So this is the last thing we kind of chat about quickly. Um, so bone marrow's disease, um is also some doin, um, it's quite common. Um And it's kind of uh characterized by your like prolonged bleeding and your um, mucocutaneous bleeding, which is basically just um, bleeding gums whenever you have been brushing your teeth, have nose bleeds. Um, it's an easy bruise and that kind of, I think, um, it's probably, um, under diagnosed because I suppose, especially if it's married, people don't really think about it. Um, when you're talking to a patient, they might, um, have, uh, you know, report that they've had heavy periods or, you know, that the bruise really easy, but they never really kind of, um, I thought more about it. Um So in terms of like kind of the what actually happens. Um So, um, bone milligram factor normally kind of s um the function of platelets. Um Well, it just helps to function rather than like the actual formation or anything of the platelets to be normal. It's just the actual just not function as well as they uh as they usually would. Um So 1 mg factor as well also kind of binds to unstabilized this factor. Uh It uh which is um uh one of the factors involved in the intrinsic pathway. Uh And that cause is on the increased a PT that I kind of match we had earlier on. Uh So, in terms of as well, um desmopressin is actually um an effective treatment at that came up quite a bit in pa I remember whenever I was starting for finals. Um and that's basically just because it stimulates um the release of factor on factory release. So it can be helpful um in patients who still kind of have um the ability to make full mi or full factor even uh they just have low levels of it. Uh So that could be a treatment option uh that uh could be uh so in terms of hemo failure. So these are probably the bleeding disorders that people are more familiar with. Um they're quite severe. Uh you can also get like an acquired hemophilia as well. Um And like autoimmune diseases or um like liver diseases, but I'm not really going to try to bite, he just going one. um So hemophilia and the are the three most common ones. Uh and it is usually kind of diagnosed, you know, pretty early in childhood. Um They are actually recessive um conditions. Um So they're more common in males. Um, so, and, uh, females are using the fact that if, um, their mother is a carrier and their father has hemophilia, uh, they'll usually kind of have a typical family history of, um, male relatives who have, um, severe bleeding or have a bleeding disorder. That was what our third year or, um, station on hematology, uh, was kind of, uh, it was a man who come in, um, to discuss that he had had um issues with bleeding. And then when you asked him, he said that he had, he knew that some, he had a bleeding um condition um run in the family and it affected, I think his brother and his uncle. Um So, but it didn't affect any um any female relatives. Um So that uh it's just that typical kind of history. Um So to just keep in mind that that can come up in a, as well as coming up in MC, QS and then as well, um if you're a carrier, you might have a slightly increased bleeding risk, but I can't really say that coming up in like an Oscar and MC or anything like that. Um So, uh again, as I was kinda um chatting about earlier on, so, um when we do a coag screen uh on someone uh with hemophilia, they're gonna have an increased A PTT. Um But the pro time is gonna be normal uh because it's only uh the intrinsic pathway that's affected. Um So hemophilia A is associated with your um uh deficient um factor eight and then hemophilia B is your deficient factor nine. And uh so you would need to kind of uh whenever we did the OSC as well as taking the history, they then give us just a clot and screen to kind of go over and it showed um that the PT was normal and the A PTT was increased. Uh And then I think it said that like, um whenever they did a clot and factor assay, um I think factor it was decreased or something. So you were like, well, the um the diagnosis here is he a uh but if they don't show you that, uh I don't really think you can tell from just, you know, having an increased aptt um walk any hemophilia that help. So, just in the off chance that, you know, we think it is maybe a hemophilia or it's some kind of clotting disorder and they've just given you um just purity results that says there's an increased A PTT and a normal PT just ask for like a clotting factor assay or something or say that you would like one just to kind of figure out um more about what actual kind of hemophilia it is. Um But again, they gave us um what factor was uh kind of deficient on the actual blood results that they gave us. So if it's going to come up for you then probably do the same thing. Um So the kind of symptoms depend on um what mutation. Um The actual patient has. Um But generally, um it's associated with really severe and kind of spontaneous bleeding. It doesn't, it doesn't necessarily have to come after trauma. Although after trauma, um they tend to obviously of um kind of more prolonged and um more severe bleeding. Um So the painful, um, hemarthrosis is kind of like really um characteristic of hemophilia. So, um basically, they kind of spontaneously just bleed into joints and not gonna obviously cause like a red hot swollen joint. Um So that's potentially just something um to keep may whenever you're um thinking of differentials for um or a red swollen joint. And uh so a lot of people with hemophilia because this happens kind of so often, um will actually have quite a lot of problems with their um joints typically affects the knees. Um So they're more prone as well, the Hema hematomas and then it can also present with like gi bleeds, um uh intracerebral blades, hematuria, that kind of thing. So basically, um any or that you can bleed from, um but it tends to be a lot more severe than the bleeding that you would see in like Miller disease for um for example, so just for completeness, um I don't think this is I've ever seen a question on this. Um I don't even know if Pome covers it. Um but uh there is a hemophilia c um just in case it ever comes up. Um So it's rather than being excellent. Um it's autosomal recessive. Uh so it can affect males and females uh equally. Um So uh it results just under your factor 11 deficiency. So your APTT again will be, will be prolonged. Uh and then if you had a clot factor, so it'll just show um kind of low levels um of factor 11. Um So it's actually more um it's kind of the exception to the rule of hemophilia, a hemophilia baby and associated with like really serious bleeding. Um hemophilia a actually presents kind of more like bras disease. Um But again, I don't really think you need to know an awful lot about it. Um I don't think I've ever seen it come up in like past man or anything. Um So that's just a wee summary kind of um just of um the um the main kind of bleeding disorders that you need to know. So hopefully, um that's kind of just helpful for your for revision. So, thanks very much everyone we got through it in the end. Um So hopefully that was helpful. Um And if you have any questions or anything, um my email is on the front of that side. Um So you can go ahead and like email me whenever you want. Um I'm in Derry um as an FC and then I'm up for F one as well. So if you ever need um any help with anything, then just give me a shout. So good luck with progress test and everything. So these will all be gone. That's great. Thank you so much, Nicole. Um I popped the wee feedback form into the chat there as well. So if you fill that and then you still be able to get the um slides through as well and if anyone's got any questions you can put on. Thank you very much for coming in. Thank you again, Nicole. Yep. Thank you everyone, Cheryl before we, um, if anyone wants to answer any questions, but thanks very much for listening and giving up on your, um, on your Thursday evenings.