Computer generated transcript

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

Because there's quite a lot of things to get through. Um And I don't want to keep you guys too late on a Friday evening. Um As I said, if you have any questions, please feel free to just put things in the chat. Um I think things are more, are better when they're interactive. So um for the questions as well, if you want to try putting things in the chat, there's a no judgment zone, just try your best. Um But yeah, we will get started. So uh just a little bit about me, just finished my finals. Uh I found out that I'm going to the Northern Deanery yesterday, which was actually my top choice. Wow. Um Just in terms of uh if people have uh any questions about that, I'm also doing my elective in India. Um So yeah, feel free to email me, message me if you have any questions about those things. So on to what we're doing today. Um I'm covering today, um your myeloproliferative disorders, leukemias, lymphoma, myeloma, and then we'll check in a bit of obstetric hematology at the very end as well. Uh The first four are your kind of big ones. Um, and then in heme two, they'll go over things like anemia and myelodysplastic syndromes. So to start off, um, if people want to, uh, have a go, I'll give you maybe 30 seconds just to have a look at these questions. Um, and have a think about what you think the answers might be, feel free to put answers in the chat or just, yeah, it makes it a bit more interesting. OK, while you guys are thinking, we will go on to the first thing. So, myeloproliferative disorders, like most of hematology in this lecture, generally, everything is gonna be classified into different things. I think there is a really nice table um to generally kind of give us our broad classifications. So first of all, we split polycythemia or well, we'll talk about polycythemia first uh into relative polycythemia and true relative is generally caused by uh things that are going to reduce the plasma volume, which makes it seem like there is an increase in red blood cells. Uh And we'll go through that. I outlined some there. Your true polycythemia is an actual increase in red blood cell mass. Um And we can split that into your primary um and secondary as well. Uh And then within secondary, we've got other things as well. So we'll go into polycythemia first. So your relative polycythemia, as I mentioned is generally a reduce in plasma volume. Um and it's generally also non malignant. So some things that can cause that. So, being dehydrated, which would make sense if you're on diuretics or if you're got diarrhea and vomiting, all of these things are going to reduce the amount of fluid you have in your blood. Uh and then make it seem like you've got an increased hematic rate of red blood cell volume. Your true polycythemia. On the other hand, is an actual excess of red blood cells and we can split that into primary. Um So that's your myeloproliferative neoplasms or disorders and secondary, which is non malignant from a hematological point of view, but can still be caused by malignant conditions. Um So going onto that, we'll start with the secondary. Um when we think about secondary polycythemia, we can split that into uh well, we can look at the um epo which is the um erythropoietin or what stimulates production of red blood cells. If it's an appropriate increase in epo, that means that there's something causing the increase in red blood cells. That is a physiological response. So things like that, it's generally chronic hypoxia. So, being in a high altitude, um if you've got a hypoxic lung, so that might be through CO PD or other chronic lung conditions, having cyanotic heart diseases or having high affinity HB which will go on to more in uh heme two, the other side of it would be inappropriate increase in epo. So where you don't have anything that necessitates having more red blood cells. But for some reason, there is an increase in that hormone and you're getting a polycythemia. So, um most commonly things like a renal carcinoma, which makes sense because epo is produced in the kidneys. So, renal carcinomas, renal cysts and hydronephrosis can cause that. Some other tumors kind of weird and wonderful things. Your liver, lung and uterine myomas is one of the things that can cause an increase in EP O. And another thing to remember is uh kind of blood doping. So, uh athletes who are trying to increase their oxygen delivery might use EP O. in that case, again, it's an inappropriate increase. Then um if we come to our primary causes, uh again, we can split this down into your Ph which is Philadelphia well, stands for Philadelphia chromosome negative and Philadelphia chromosome positive. So your negative ones uh are the the three most important ones that you need to learn are polycythemia, vera, primary myelofibrosis and essential thrombocythemia. A uh Philadelphia chromosome positive, primary myeloproliferative disorder is basically just chronic myeloid leukemia. I just found that it was good to get your head around how things are classified. Um and where everything fits in. So we'll talk about uh uh negative ones first. Uh Before we go on to that, uh this is something that's mentioned in the lectures you might have already done this, but I think it's a useful thing to get in your head. Um Generally, these disorders are issues with proliferation and maturation and you can go into the specific spot, whether it's a type one mutation or type two, but you can kind of read up on that. But I think in um a normal progression of kind of hematopoiesis would be your the blast or your blast cell represented on the left hand side, going to a mature cell. So you have proliferation expansion of your cell um and also maturation. So going through the process from a blast to a mature cell in myeloproliferative disorders that says in the name, there's a excess proliferation. So you have too many cells but these cells are still mature, so they still will perform their function. But you get the other issues of having too many cells in different places in acute leukemia or acute conditions, you have both things that have gone wrong. So there's excess proliferation but also there's proliferation of immature cells. So they won't actually perform the function that they need to. So having said that we'll go on to our er ph negative myeloproliferative disorders. So first one is polycythemia vera. Um in terms of it very classically, you'll have kind of itching after a hot bath. Um you might also get peptic ulcers and the other side of it is things like headaches, increased risk of thrombosis, blurry vision and lots of other things. And you can think about presentation with uh two different characteristics. So, histamine release excess, histamine release. Um, and that's causing your itching, uh, pruritus and then hyperviscosity because you've got too many red blood cells circulating in the blood and that will obviously increase your risk of things like strokes, uh, th thrombosis and headaches as well in terms of your investigations, uh, more basic investigations. So obviously you'll have an increased, um, hemoglobin, increased hematocrit. So you've got a bit higher red blood cell mass. Uh, and there's some numbers there as well. You might also have increased platelets and white cells. But that's sort of by the, by the most important thing is that you've got an increased hematocrit. And importantly, uh, you might have a, you'll, you'll have a reduced serum epo because you've got excess, um, red blood cells. So you'll suppress that hormone. Uh, and then I've put at the top there, the jak two mutation. So that is a really important one. It's a sort of buzzword thing that you'll see in, um, your path exam. If you see that mutation, you ju you know that it's probably going to be a myeloproliferative disorder over any other, uh, condition, secondary condition in terms of treatment. Um, you can split it into two different things. So kind of addressing the problem reducing the hematocrit. Uh, you can do things like venesection. So, basically doing therapeutic blood tests to remove the blood from their body. Uh, you can also do, um, use hydroxycarbamide, which is something it's cyto reductive. So it basically prevents or reduces the production of cells. You also want to reduce their thrombosis risk because they've got so many extra blood cells circulating. Um And with that, obviously, you're controlling the hematocrit by doing the other things. Uh but you also might use things like aspirin and you want to aim to keep their platelets low as well. Essential thrombocythemia is the next uh disorder we're gonna look at in terms of presentation, It's very similar. Um, but the main thing that we worry about is a risk of thrombosis, both arterial and venous. So, strokes, tia s um kind of thrombosis in the limbs which might lead to gangrene in the lungs, pe and, and so on. Um, there is also a risk of bleeding because of thrombosis. Obviously, you're depleting your platelet sores. So you might also have bleeding and you might get a modest splenomegaly because there's basically clumping and um, sequestration of your your cells in the spleen and the symptoms of your hyperviscosity. Similarly to uh polycythemia, increased headaches, lightheadedness, blurred vision, and those kind of things, your investigations fairly self explanatory, you're gonna have increased uh, platelet counts. You might also have increased other uh myelo