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now. Okay, One welcome to talk on Hemoglobin Emergency by Dr James Clot. Find yourself seen. And along with sterile, we will leave it that he would hold you team here. A mother, please. Um, incredibly lucky to have to do is start with us. They do in the presentation. So, like a pediatric, he was called me Trading. Currently working out great on the street. Uh, please have any questions you have during the talk to the chat. And if you have time again and we'll start the clock to, um and attentive these whole days, if you want to stop it and go ahead. Fantastic. Thank you so much. Stuff in and they're having me. A policy is slightly delayed. Start everyone. Um, so I've been asked to speak on him Political emergencies today. Um, and hematology is a little bit of a black box, Many people. So I want to make sure that if you have any questions, do ask him in the chat, and I will try, um, address. I'd much rather have a meaningful chat about, um, one case than you know you to blitz over and not understand several. Um, and also I'd like to think if there's any cases that you've come across in your training or the last few medical school about that you have any questions about you wanted Teo delve into a bit more detail. We can cover those at the end. It's always nice to hear your experiences and try understand those reflect almost Well, okay, so we're gonna cover some urgent, urgent sort of complications and presentations off him for a logical conditions, looking at how they present on why they present and look at the sort of emergency actions that need to happen on when to seek help on that maybe straight away. Or it may be that. But we always know that although there is always hedge in etty in people's responses and everything else hemotologist the whole are ready to be friendly bunch with much rather hear about patients on say, that's fine. Carry on them, not hear about, um so please always ask for help If you're not sure. Okay, Eso we're gonna look at a range off clinical case is now. I'm not expecting you to know the answer in any way, but I hope that you come away from this dog having a little bit more confidence with some of the common presentations and things to look out for is just headlines of Remember this. That's big. That's a big headline. Have to go looking for. Okay, So our first case is a 54 year old woman who presents with a new diagnosis of a high grade lymphoma. On day she received one called R Chop chemotherapy seven days ago on, she presents to the E. D. The emergency department with a fever off 38 degrees Celsius. What complication do we anticipate now? I'm not going to pose any hematological knowledge on anyone here. So, um, do chip in if you have any thoughts, but, um ah, high grade lymphoma is normally meaning it's ah, rapidly replicating human logical malignancy of affecting lymphoid cells on high grade means it's It's normally quite aggressive, and so it grows quickly on needs treatment quite quickly, as opposed to a a low grade one, which is a bit more indolent and doesn't always need immediate treatment. Our truck chemotherapy is the standard of treatment for most high grade. The lymphomas Um um, the worry of seven days in is that it is quite myelosuppressive Um So what that means is that the bone marrow is suppressed, meaning that they can become anemic thrombocytopenia, meaning that platelet count is low. And also they're white. Cell count could be quite on the differential there is. You know, if the white cell count is that you have neutrophils lymphocytes and a few others, but predominantly you have a neutrophil count or and which is low, which is a neutropenia. So we're expecting them seven days in until about they 14 to be neutropenic. Um So the thing we're going forward here is that they are attending with febrile neutropenia, which some people use a short hand. They're saying they're neutropenic sepsis. Now the worry here and forgive me for being a little bit more didactic and teaching. This is so complex in many ways, but virtual teachings quite difficult to go through these cases in the inside of a backwards and forth fashion. So in this circumstance, the worry is that they don't really have much of immune system to speak off to fight off any form of infection. So what would be a a relatively trivial infection is much more likely to be bacterial on Also, that bacteria infection is much more likely to become disseminated on widespread. So a full on septic picture, as opposed to us of localized infecting their neutrophils, aren't able to hold onto the infection to stop it spread into the bloodstream. Um, and so you can become life threatening much more quickly. So this is one of the common things that we see in people. Patients on chemotherapy. Um, Andi, we worry about it a lot. So whenever we counsel patients starting chemotherapy, who we expect to become neutropenic, we will say to them, Right, Any temperature over 38 so straight to an E on many trust will have a ah, quick passport or a card or a letter that the patients were having hand to the triage nurse, saying, This patient is on chemotherapy. You know, if they present with a fever, immediately give thumb triggers and quickly get antibiotics in within a golden hour because we know that that can save lives. And it's been demonstrated that the quickie antibiotics in the quicker you can offset and mitigate that risk. Um, so the first question here is what antibiotics will you give? Um, and this is very much trust dependent. Your trust will have a local neutropenic sepsis protocol or February neutropenia. Particular called it one of two things on deviously. Just to clarify. There you can be neutropenic and febrile on, not have neutropenic. Sepsis is, but you treat has not been exceptions, because if you have a trip, you could be separate from a viral infection on. That won't be a septic picture, but you don't know at the outset where they're going, so you treat house. Need to be exceptions on. Quite often you will give broad spectrum antimicrobials, including something like has a sin most commonly on. But what's, um, Gram negative cover on? The rationale for that is where the bacterial infections come from. In neutropenic patients, the neutrophils actually have additional role in maintaining mucosa membranes and maintain the integrity of those which is why they were quite new. Medications were quite often get or um or or a lot of mucosal ulceration. And they'll also get potentially some colitis type picture where the gut mucosa is also less protected and becomes quite afraid for one. A better word on do that barrier, but they normally have in intact to encapsulate the bacteria within the gut is less intact, so you'll get what's called bacterial translocation. So the gut bacteria, which most commonly gram negative bugs, will translocated into the bloodstream or elsewhere and cause a significant infection. And you're much more likely to get gram negative. Sepsis on that comes with more likely to go into septic shock. So and so that's why I quite often you'll have tablets in for, you know, broad spectrum cover up with something like gentamicin or something similar to cover that type off infection to keep you safe. Um, so that's why we you you're pretty close to incorporate that when they come in, we want to know what they're growing. So you're immediately treat down there, sort of. You know, the old fashioned septic septic six line and you'll get some blood cultures off. But also you'll make sure you get a little cultures off that you possibly can. The worry here is that quite often you won't find a clear clinical focus sometimes, you know, but because you don't have neutrophils, you won't get localization to where the bacteria is. So if they have ah pneumococcus, you won't get ah localized pneumonia because there's no neutrophils that, you know, former focus there, so it becomes quite widespread quite quickly, so a chest X ray quite commonly won't show of focus. Same for UTI. So you don't have neutrophils toe on your urine dip to come up with a white cell positive. And if you have a non nitrite forming bacteria, you won't get nitrogen, you know. So make sure you send off a year and a swell my cross. Be culture and sensitivity. Make sure that in today's world, use end of Kobe, PCR and various other viral screens. Get the chest X ray even though it may, it may not show anything. If there's lines, make sure that you get paired cultures from the line on drum the peripheral blood so that you know where the infection is coming from on. Do you're going to treat him quite aggressively. Okay, the last question is a little bit loaded in the sense of when would you consider something called Granulocyte Colony stimulating factor? This is a subcutaneous injection that will stimulate granulocytes normal to mobilize from the marrow toe, increase the peripheral circulating neutrophil count to allow you to the person to better fight off any infection. In this case, we've got a lymphoid malignancy. So it would be entirely reasonable if they are neutropenic to give them some Jesus F if it starts appropriate. There are certain caveats to that, um, such as Hodgkin's lymphoma and also specifically myeloid um, malignancies. So if you had a patient, we have something like acute myeloid leukemia. You would be very reluctant to give them that because it might just drive the underlying malignancy and make it worse. So you would potentially not be thinking about that in my lawyer malignancies. Which is why, if you're not sure, always have a chat to your friendly neighborhood hemotologist. Okay, so hopefully that's a very common case that you probably will see a very commonly if you're clocking in any because these patients will quite going to come through there. And it's just about making sure that you understand that there is a time sensitivity here to make sure that you get the appropriate cultures off on start urgent treatment. Okay, Hopefully that makes sense. So we'll move on now. Two case, too, which is a very different um so in this case, we have a 25 year old woman Onda. This is another emergency department presentation. She comes up, rocks up with a fever and headache on they do a routine for blood count, which shows a significant thrombocytopenia off only 15 times tends to nine, which is really significant on acute thrombocytopenia comes with a really wide differential on there. Only a few life threatening causes. Um, Onda amongst thumb would be things like cute leukemia. Um, things that i t p but given the presentation, the other thing that we might want to look at is something called a micro angiopathic Humility Can Neemia, which can come with Thrombocytopenia Onda? That's what this case is alluding to because the lab call up and say the hematology registrars on the way in the film. But we think we can see you something called schistocytes on the blood filled on this Would This is what they look like. They're you can see. These are in case you don't know what a peripheral blood film looks like under the microscope. These the red things here are red cells which are not looking in there classically normal way in any way, shape or form. You've got ones which are all very which probably campsites. You've got some chest sites and some throat sites in there is where which don't have the central palate. But you've also got these a road cells here which are schistocytes all fragments. And there are literally red cells that have been shared, shared in half, which can come from, ah, multiple array of causes. But this has, ah, some emergency differentials. The one that we're thinking about here is something called T t P from about IQ thrombocytopenia purpura, which, if left untreated, has a mortality rate off around 90% within 48 hours. So we don't mess around when it comes to this one. There are some urgent investigations that we would send off, which I'll talk through in a second. Um, but the mainstay of management is removing is treating emergently. So this is one that the hematologist would be very, very interested in. Want to be there the manager and transfer toe appropriate tertiary unit for a proper treatment? Um, thank you. Yeah, that's a really good point. The I see can present with, um Besides, Pina can present in this way and can present with fragments as well. Um, but, um, you'd also have, ah, clotting screened arrangement. So that's why other investigations that be useful here would be one to repeat your full blood count to make sure not missing anything but to a clotting screams Well, to make sure that we're not missing anything but the I see you normally have, ah, more of a clue from the clotting parameters. But also, you don't get quite so marked a thrombocytopenia, um on. Given that the patient presented with fever on with headaches, it's It's alluding to this thing called TTP pen tap, which you know is less useful because actually, they don't present with this. So it's a bit of nebulous thing, but classically present with anemia. Thrombocytopenia onda the underlying mechanism off This is basically with the next like to show you this lack often enzyme called Adams ts 13, which in health allows very long thrombotic multi men's off willebrand factor to be cleaved so that they don't end up in a big clumps. And if you lose that deficiency normally in the adult population, because you end up with an antibody attacking the enzyme, so you end up very, very low or in Children. You have a congenital deficiency, which is made worse by an acute sort of events, like a bug or something else. You end up with these big plugs off uncleaned from willebrand platelet from from by, which clog up the very small capillaries. And when you've got that clogging up, the red cells can get through a literally tour in half. Um, on Do that, those clumps conclude in the brain, in the kidneys, in the heart, the lungs and elsewhere on. That's why it's life threatening. So you want to get the diagnostic sent off for the quantification off the Adams ts 13 enzyme, but also the antibody against it to confirm the diagnosis. You can manage this by managing the underlying giving FFP, which contains some Adams. Two years 13 on There is a recumbent Adams TS 13 on the horizon. But the mainstay of management in current practice is very urgent. Plasma exchange, which basically means that you're going to put their blood through a machine to get rid of all of these clumps of everything else and replace with basically softened surgeon FFP back into circulation toe get to reduce her basic increase the Adams ts 13 s and then go from there on. Also, in the case of a quiet TTP start something like steroids on do certain monoclonal antibodies, which I won't delve into now. But mawr directed therapy Teo mitigate the underlying autoimmune cause toe try and increase this going forward and we go to give things that reduction happen, things like that. But this is one of those things to be aware off that will have, you know, the hematology team coming running to the any to sort out and send off because it is so life threatening so quickly. But the difference is quite broad of, um, our heart including, you know, help syndrome in a pregnant lady, which is what 25 year old females about that case to make sure you do a pregnancy test, making sure that you think about the ICS and looking at the clotting profile, making sure you look at a typical aches us and and typically at us as well, which was the same way. So renal function, things like that are quite helpful to delineate. But yeah, the life threatening thing there is TTP andi to be aware of that, you know, understanding of Muhanned Schistocytes. And why? Why, That could be life threatening. Um, moving on to our next case, Case three, which is a 27 year, seven year old male who rocks up to the emergency department with fever and bruising. You might notice a trend here on, but he has a bit of ah pancytopenia. So just to be really clear about definitions, you have basically three lineages off cells your red cells, your white cells and your platelets on there, the three lineages. And so you have ah, single cytopenia, which is just a name of that in your or leukopenia you have by cytopenia, which is one of those two of those and then pancytopenia, which is a three being suppressed. Um, and you also have, in this case, a coagulopathy on the lab call you up. So again the hemotologist on the way in. But they think they can see blasts on the film. I'll give just 10 seconds for people with chapters. Anyone have any clue what this case is hinting at as Teo Very concerning life threatening pathology, That's we would be wanting to treat very aggressively. Absolutely fine, not to know this. Okay, If there's no takers, I'm going. Teo, move on to yeah s. Oh, well done. You're hinting they're at using the acute leukemia? Absolutely. So acute leukemia is very likely in this circumstance. And it's very difficult sometimes to distinguish between myeloid and lymphoid without knowing that there is a hint in this clue as to what were hinting at a zoo. Well, with the coagulopathy. So this is a blood film on again. We've got the every lab has difference. Slight difference. Stay in certain things that vary. But these cells in comparison, these very stain cells are very large in comparison to the red cells room. So these are very large cells. And if you look, they've got the nucleus, which has a very diffuse pattern. So there's no clumping of the chromatin within their within the nuclear. And there are some nuclear. The weather's been empty areas on diffuse cut this middle cell. And so this is This is very clear, immature cells after this up last, and it's got a quite a specific morphology here where you've got what's quite nicely turned off the bat XL, where there's like a bi lobe thing going on And this cell in the middle in the cytoplasm, Lots off rocks. In fact, so many was packed with our rods. Now, this is what they look like. Onda these bundles off rods here, um, our pathognomic for a myeloid malignancy. And these cells being the buttock cells with all these things with a coagulopathy is a particular type of acute myeloid leukemia, um, which is called acute promyelocyte IQ leukemia or a PML, which is a very interesting subtype off myeloid leukemia. Onda is classically associated with our meds. Earlier comments off disseminated intravascular coagulation said the clue in the first question was that there was a quick allopathy Now, given that sometimes it takes a while to get the film looked out. If you suspect leukemia, one of the first questions hemotologist will ask you is is there any quick allopathy because we're worried about this complication? Okay, Is any, um, leukemia can technically present with regulation derangements. But in this circumstance, a PML pretty much always presents with D I see on do the, uh, a female has a really good long term prognosis. Talking 1995% plus on D can sometimes be treated with, you know, eventually non chemotherapy related things. Uh, but that prognosis only applies is if the patient survives the first 2 to 7 days because the coagulopathy is the most likely thing to kill them on. You have to be really aggressive with coagulopathy management. So it's not that comments quite rare one. But it was in 5 to 10% of young adults off leukemia. Off meloxicam is our this one, and it's highest in the younger people. Um, it has this just a help guide you. There are various ways of characterizing leukemia that's normally if you look on the right here on where the cells have gone wrong in their differentiation from stem cells down. Teo, you know they're professional sell at the end. The neutral off other myeloid cell on this one is the promyelocyte IQ, so it's one of the progenitors that is a little bit after the stem cell stage. Onda. The morphology used to be terms of em one toe seven. Based on where this differentiation point happened, that's gone a little bit out of favor, but you may see that terminology around, so it's just to explain that a little bit has Ah, simple translocation. She's turned PML rara, which is basically the retinoid acid receptor. So the treatment is really interesting because this translocation basically stops the promyelocyte differentiating down the line. So we give them plenty of retinoid acid. Uh, which is Ultram is with no tattoos or on that allows the cells too rapidly differentiate which comes in zone complications. But that can be life saving very quickly, but can make the coagulopathy work. So what we're gonna do is diagnosed with a blood film will try and do a bone marrow. If the coagulopathy will allow us, we'll get the rapid diagnosis. Absolutely, President. Treat without atra. Um, Andi, get the rapid diagnosis confirmed Diagnosis. Normally by realty, I'm PCR, which could be turned around normally if you ask for urgently within about 12 to 24 hours, depending on where you are so we can get a diagnosis. But you're going to stop aggressive treatment based on the the morphology because it's diagnostic with that film we saw before. Um, so yeah, as we said, a PML with the I see. So we're gonna manage to the I C, which is the life threatening complication here, which comes with a range of things that the icy can happen secondary to all sorts off pathologies. Okay, on what it basically means is, you know, something quite nasty has happened within the circulation, but my professors used to use the old adage you may have heard, which is the I. C. Stands for Death is coming because it's such a life threatening complication, and it can happen. Second week of September is it can happen secondary malignancies, but most commonly infections or just being very, very poorly. And it's basically out of control hemostasis, where there's consumption off along the platelets on Do the clotting factors within one area. So there's uncontrolled clotting on doesn't leave anything leftover elsewhere in the body, so they can have bleeding alongside it. Thankfully, they normally present with one of the other phenotype, but it can be very difficult to manage on. We often have to be very responsive to how the patient is clinically, but normally it's supportive care. So keeping the five British and up because that's consumed very quickly, keeping the clotting screen on with fresh frozen plasma, trying to keep the platelet count relatively normal, it's very common for, um, people with three. I see pretty with a PML to have subdural hemorrhage is just one day cycle. It's like headache, and you scan them that got subdural hemorrhage. It's very difficult to manage that, Um, so you're just trying to keep up the process so quite often they'll have three times a day clottings. And for blood counts and regular products, you have to manage the fluid overload, and it is difficult there. You also manage, you know, they often get a fever, and you have to regard them as functionally immunosuppressive cause they have no neutrophil. So you have to manage them for nutrient. Accepts is when you get a manage your license individual coming to shortly on, we're also gonna manage them for other complications. Um, so in a nutshell, that's a p M. L. D. I see. Basically, we've talked through as a med referred to it. We can get schistocytes in 30% of the I see, but it comes with quite a marked clotting derangement, which gives you the big clue what's going on. And there is a d. I t. Score in temperate out there to help guide because it can be difficult differentiate between them, but it's just trying to keep up with, you know, the supportive care where you treat the underlying cause. Basically. So that's how we treat that one. Does that make sense of hope for everyone? Yeah. Um, right. If everyone's happy with that, um, do interrupt with questions. If you have, um, we'll move on to case four, which is Ah, 40 year old woman who presents again to the motive department. With fever on this one has shortness of breath on. Do they do a full blood count on? She's anemic with a white cell count of 120 a platelet count of 35 on the chest. X ray is performed, which shows disappearance here. Now I'll just give you 10 seconds in the chapters. Anyone have a spot diagnosis of what they think might be going on here in terms off v acute complication off. Ah, previously unknown patient to hematology. It is a little tricky, this one, because obviously there's a wide differential. But this checks X ray shows quite, I think. Good. Good thought there was definitely a meniscus there, but I think that's actually just where the fissure is. This is on on this. This. You can still see some lung markings on the right base, then maybe a little effusion on the left there. But this is very marked infiltration, particularly on the lower basis showing, you know, this could be an infection or anything else, but given the very high white cell count, you're very worried about Lucas Stasis here. Newman is definitely one up there, but given the high white cell count, you're worried this is infiltration off off those white cells into the lungs. Um, on go. You wanna understand what's going on with his very high white cell? Count on. Obviously, you're gonna treat thumb down various lines to make sure you understand what's going on. Um, so this very high white cell count of 100 twenty's the clue here. And so if you have that sort of level, like, you know, more than 50 you have to start on the short of breath. You have to think about this whole thing. So you want to get a blood film to see what's going on on this one will show up in Acuna of some sort on. It's much more common in acute leukemias. With those blast as opposed to the chronic, leukemia is now just to differentiate there. The acute leukemias are the different. The differentiation point of them is normally the stem cell. Or they're very early precursors, where you present with the blast as a poet, and so they because the stem cells rapidly replicate they, the symptom onset is quite quick, which is why they're cute, whereas the chronic leukemias are normally the professional cells. So stuff like the lymphocytes or plasma cells, or the neutrals, which are replicating much more slowly. So that's why they're called chronic was often the symptom. Onset is is much more slowly because much for indolent on go, that's a good way of understanding why they're acute versus chronic. On it comes to where the problem of differentiation tree has come from. To lead to that in increased cell production on blasts up as we saw the four big cells and particularly myeloid cells big and sticky on so they will clump and cause, um, you know, congestion wherever they are, there are actually problems that you can get with any cell line being increased, So if you have a very elevated red. So if you have, that's a polycythemia with the very, very it's hematocrit you can end up with hyperviscosity. If you have a very elevated platelet. Counts are thrombocytosis. They can also come together, giving you some other problems with that over that very small so you don't really get sort of viscous symptoms. And if you have problems with lymphocytes, which produce antibodies so stuff like immunoglobulin producing liver prolific disorders or particularly myeloma that can present with very, very high levels of immunoglobulin, which can also cause hyperviscosity symptoms and that basically means that there is sluggish flow on def, there's lots of Lucas status. You get similar picture where wherever there are small vessels, you will not get good flow, and therefore you'll get an element of ischemia so you can end up with cerebrovascular accident scan fusion. Without clear evidence of in fact headaches, you can end up with poor Marie complications of dyspnea risperidone alkalosis. You get that with cardiological intonation, angina and even, um, I've So there's a wide array of ways that Hyperfocus is leukocytosis can lead to symptoms on that when you are symptomatic, you have Lupus Stasis. It's most common when you have a white cell count more on the 100 but it can occur at lower level. So have a load index of suspicion. For this on the way forward is tow. Try and dilute things down down to allow better flow so fluid, like aggressive, fluid management. Sometimes we'll do something called leukapheresis, which is similar to the plasma exchange where you're gonna try and, you know, Betty, put them through a lot of effects. You have a pheresis machine toe. Try and sir, spin off some of those white cells and put back the stuff that you want in the circulation. But the mainstay of management is getting treat is treating the underlying course. So that's site or reduction. So really gonna give definitive chemotherapy if it's acute model Kenya. But sometimes if you're struggling, you might give something like Hydroxycut mind, which is a global martyr suppressive. Just bringing the countdown toe reduce symptoms, um, but depends on the underlying course, so that needs evaluation by the hematology team. It's a bad sign if you have Lupus Stasis. Your mortality is, you know, close to 50% normally within a week, so it's in needs Sergent Treatment, not least because it can kill them off itself. But it's also a sign that the underlying cause is quite marked on can be a later sign. It doesn't occur very commonly, but when it does, it could be quite scary because it takes quite a lot of aggressive management to get things under control. And you're managing not only the complications but the problems of therapy so fluid overload their hearts, maybe difficulty in the struggling with the producer just infraction. It could be very tricky. You put some very good thoughts in your thing. They have a plural fusion because it does look very congested on you might well do a CT just to evaluate that, because if they're going to drain off to happen, breathe better. If they're really pack of near can oxygen and you want to drink it off, they will end up with malignant effusion as well. So sometimes that could be helpful, and you will be treating them for pneumonia. Concomitantly. But this is what this case was driving at this overall picture of Lucas Stasis, which is quite a scary complication, right? Uh, Case five. Um, so six year old book on the medical team are fast, bleak to review at three in the morning on the ward following us. Ah, full or a single episode going entirely clear, Um, and I've done these points in the wrong order, and I apologize. He's been recently started on chemotherapy for bulky D L B C l um, which that's for diffuse large B cell lymphoma. This is one of the high grade lymphomas that we have in case one and bulky means that it's big and mineral means more than a limp, a lymphadenopathy little mass of around 7 to 10 centimeters. So it's It's a big, massive tumor, Um, and he's got a background of the age of 60 of a bit of chronic kidney disease. But blood have done urgently, and his is his office. Baseline has gotten a K I. His potassium of silly high, and also his calcium has dropped massively on. When you examine him well, he's got a tachycardia on someone's flailing around for any CG machine trying to evaluate what's going on. Does anyone have any thoughts about what we might be dealing with here? Spot on Stephanie um, TLS tumor lysis syndrome. Um, he depending on where you work, sometimes emergency therapy needs to be started in a in a non some of hematological contacts. Or sometimes, if there's very bulky disease tumor. Lysis can even be a presentation symptom and presidential off some bulky disease where the there's auto license. So what we're getting at here. So these are the questions I have. It was a different you'll be most concerned about, and prediction of who might get this on treatment will go through that now so it could happen in a range of malignancies. But it is most common in high grade hematological cancers because there is quite a lot of tumor burden on. But when you lies a cancer cell, most commonly a cute, cute lymphocytic leukemia, high grade lymphoma searches Burke. It's all diffuse large B, particularly if you've got a big disease burden, bulky disease. Then those cells get destroyed either by auto license. So if you got a big mass that suddenly he's got very poor angiogenesis of the blood vessel formation is pretty shocking. Then those some of some of those cells part that bulking mass will just die because they're not well perfused on you get release off all the cellular contents, which includes all the DNA metabolites and periods and everything else Your phosphate, your potassium and all the cytokine is well will be released into circulation. On that can happen after chemotherapy as well, particularly in someone who isn't able to clear those very well. So if you got underlying renal impairment and most people who are a bit more elderly, so if you're 70 and you've got this and you would be much higher risk. So if you had a 70 year old and CKD bulky mask, you know you'd be sat there going Oh my God, you're going to get something and you do everything you can to try and risk mitigate that. And there's various mechanisms by which it happened. So, you know, everything gets a Lowe's period. Metabolites get broken down into uric acid on do it could be quite difficult to treat that because your Cassidy's requires functioning kidneys and urinary excretion. And if you've got a lot of it, um, or you've got, um, poorly function kidneys, you're going to get overwhelmed. And if the kidneys aren't clearing stuff on, you get basically uric acid crystals formulation and then that potentiates phosphate crystal deposition. In the real troubles as well on, do you get a burden of potassium's well, so you end up in a significant dysrhythmias, so they're quite they can go into V T, and it could be terrifying. And that can then make the AKI worse. And that can lead to other sort of deterioration of fluid status and poorly functioning. So it is a very scary thing when it happens, some of things that we can use to mitigate it is on the allopurinol, which is something oxytocin, the hip It er on basically doesn't allow xanthine to be changed. Your gas is, which means it could be just basically, you can just clear it from from the blood of it more quickly. Or you can use something called respiratory, which converts your gas in the island. Toying, which we don't, is a step that some mammals have, but humans don't Teo help clear it off a bit quicker and meet basic tenets that won't clog up the kidneys effectively. Um, so your kids can be used the prophylactic measure and can be used as a rescue med ality as Well, um, and we have two ways of defining Gemmell. Isis one is basically on lab criteria, so they they clinically well, but the year it goes up the foot potassium goes up the foster, it goes up in the calcium drops because it crystalizes out circulation. Um, and then you have clinical dealers, and if you're in that situation, your patients over a prognosis is bad. You know, you need to be on top of this quickly on, not least because it's, you know, a k I, you know, cardiac arrhythmia, seizure or death, you know, is a fairly catastrophic event here. And so if you have that situation, we're going to clinical TLS you're gonna be chatting toe. You know, I t u to get them on the filter to try and clear this off on going to get that potassium under control, get the uric acid of getting on respirator days and try and balance this. But mortality is very high if they end up there. The identification of those who are higher risk has meant that they could be pre faced with allopurinol. It takes 24 hours to work or given prophylactic respiratory Azor rescue rescue okay, is to rescue situations. So I mortality from TLS is actually much less than it was 20 years ago. But it still happens, and it can be very rapid. Ongoing. Have a completely well individual who starts chemotherapy and is dead within two hours. It could be that quick. So quite often, after starting a high risk regimen, you'll be doing bloods four hours afterwards after a baseline and then every three times a day, twice a day, depending on the risk to just make sure you're not missing it. Be aggressive with fluid management. Um, so it can be quite a scary complication if you're not careful. Um, right. Uh, okay, six, which is our last case. So if I haven't covered something that you've seen in the past, then do start thinking about it now. Um, because this is a 19 year old man with known HBs s, which is a homozygous it for sickle cell. Okay, so this is sickle cell disease on do They're on a regular transfusion program showing they're quite severe. They're on Hulu doctor or the nose hydroxy carbon might to try and increase their hemoglobin effort to keep their complication rate down. So that's also matter. You're saying this is a high risk individual. There are iron kelation therapy because they're on the regular transfusion program so that I am burden may well be very high. They're on the appropriate prophylaxis with penicillin on some folic acid, which is a devious merit on They come in presenting with a one week history off generalized pain. And it's not responding to regular and it's regular or a large easy at home. And it's getting worse in the right arm and in the rib that completely, you know, without fever. The heart rate's 96 that blood pressure's good response rates 24 SATs 92 on room air on right basal crackles. Okay, Um, so, uh, any other has done a really good leap here? Um, so, yes, this is ostensibly a of the O'Dea vsc of evasion. Clues of crisis. Of those is a disease with the pain with some early signs off evolving acute chest syndrome on this patient, so you can tell this is on uploaded. Pdf causes. It was mentor evolved over time. So the initial management here is, you know, make sure that they got pregnant pain relief, You'd want to make sure they're probably in. And I have very regular obs. You want to make sure you you understand that underlying disease So you want to make sure they have a on urgent for blood count group and say even highlights of the transfusion lab that you gotta sickler in because particular transfusion program, they're very likely to have aloe antibody. So antibodies complicating giving a safe trip, cross match on if they're gonna need transfusion or exchange you. I don't know what's going on there. Um, you're going to be looking at their liver function. They're renal function. You're going to look at the infection markers. And you lost me thinking about there sickle percentages. Well, toe, think about whether you might need to do something where you're gonna be doing some very sensible risk mitigation, regular observations, particularly given them opiates, which will give him respiratory depression on. But, um, you know, they may have a ton of them is well, so you want to be observing these guys, like wants to hourly on. There is some science here. They're they're not breathing very well. And that may be just the fact that got some pain in there, rip, and they're just not breathing very much. But it could be something else. So you could be wanting to get a chest x chest X ray on you. Review them five hours later. The chest pain is awful, and they drop their stats to 80% on room air Respirator up to 33 on. You got a chest X ray back and it shows an infiltrate. So the left base with pneumonia on some interstitial static covering, you do an urgent a BG. And it's just awful. You've got a you've got hypoxia on. You've got a little bit of a borderline high res to, um on. But, you know, this is this is bad. Okay, this is on emergency, and the reason for that is the single acute chest syndrome. Okay. And this is a really simple, um, diagnosis. People always over complicated. It's basically on acute illness in a sickle cell person with fever or respiratory symptoms. One or the other on radiological. Evans eventually infiltrate. That's it. Okay. They may not be that very bad. Is obviously severity markets on, but it's the literature is mixed, but if they're high pox IQ. It's bad. Um, the mortality rates Very, um with between three and 10% mortality for each admission with acute chest syndrome on day one and five need to go to level three intensive care with inflation. So this is something that you need to escalate quickly. Um, and it can be the way that people present. So they come in breathless and you get extremely like Jesus and you put him on oxygen and you go to give them incentive spirometry on anything else. It can be when they've been in for a few days. Um, because I've had a Basically, the crisis that got pain means you're not breathing very well, and this comes down to the underlying mechanism off sickle cell disease. You get an initial insult whether that be, you know, it's a hypo perfusion or infection or infarction, and you get reduced oxygen in the chest, which leads to HBs. Polymer ization. Reduce per pump, the blood flow more vasal occlusion, more high pox here. More promise. A shin and a big vicious spiral. And these individuals can deteriorate so rapidly you can have someone who's completely well eight o'clock in the morning on needing intubation by midday, if not sooner, because this process happened so quickly. So you. That's why if you've got someone who's high risk, you know, chest crisis affecting their ventilation they're on high opiates. Reasons for respiratory depression. Often than not breathing very well. Infection on board. You know, signs of fever or breathlessness. You want to be aggressive. You're going to be wanting to involve a pro professional. So you want to make sure that you examined regularly. Get a get the HEMOTOLOGIST involved. Get the outreach team involved. Get the I. T. U teams involves Give them from prophylaxis. You don't want them having a P to make this process worse. Hydrate thumb. Get them to you. Leave me a but hydrate them aggressively. Give you antibiotics. If you know it could be a confidence infection, make sure they're not. Put their pain free. Get chest busiest Volvo and, if necessary, give the top up transfusion, which is obviously based on their transfusion history. Everything else. But you need to be quite alert to these patients because they could deteriorate so quickly. It can be really scary on that's why I want to highlight this. You know if you have Ah, a patient who is affected by sickle cell disease most of time. They come in with a simple days. Reclusive crisis. It could be quite easy. Manage with good analgesia, good recognition and good supportive care. And it won't deteriorate if you're not careful. Things can quite quickly get worse. So just being vigilant is what I want to highlight that. Okay, those were my six cases on. We got quite close to the hour times and not too bad. I hope this was the QR code for feedback. So if you have anything you would like to be back there. Be appreciated. But I would really like to you to invite you to ask any questions. Or if you got any interesting cases that we think irrelevant here, I would be very happy to chat about awful waiting for a question to come through the chart on the way. Thank you so much that I was really I really enjoyed it. And I'm sure that the rest people listening Good, too. Thank you. Welcome. Um, if you don't want to put any cases in this forum, please feel free to email me if you want, Um, always happy to respond if if that's something that you wanted to know. Okay, um, it's a very big question. Um, out of said the beginning, uh, it's hematology is one of those topics that because it's a relatively yet low yield question in the exams and everything else for medical school, most people don't really know about him for for anything. So it becomes a bigger black box for many people throughout medicine. Um, so I think the most important things to know about you know, they won't be expecting very much. I promise you that we get, you know, junior registrars and also what's coming through who genuinely, you know. No, the basics on. But that's completely fine. The If you're on the ward on a on a malignant ward, you will get involved in the malignant side of things so that the the lymphoma site the acute leukemia side on. You'll learn a lot about the complications in about the margins of patients on the ward. What you won't get really unless you go to clinic is some of the common hematological questions which you won't be what we're really appear on. The logical wards So how toe Look up and investigate and anemia or thrombocytopenia. How did you the basic work up on So that would be you wouldn't have to brush up on. But see if you can get your registrars and consultants to go through those topics that will be more useful to you If by some bizarre, weird cousins you don't end up becoming completely enamored with hematology will become useful for whatever career you end up going into. Um, because he was totally touches on all everything else. So, yeah, I think learn on the ward, Just be engaged. But try and get them to talk about things that you might might get if you're elsewhere in GP land or in pediatrics or in keep metal surgery. Have most those What are you getting treatment of? The I see. It's a very responsive thing there, Stephanie. You You basically give what you need, Teo. Quite often you will give thumb. If they're very, very from such a panic, you will give thumb platelets to maintain their plate again, about 50 if they're bleeding. If they're not bleeding, you probably won't give them platelets because you don't want to make it worse. And you definitely would avoid platelets in TTP. So you want to be confirmed that with the diagnosis, FFP is normally the mainstay of management. End up with more, more significant Ah, coagulopathy van. They do a thrombocytopenia most commonly, but it's very variable. So you are responsive to the individual. Okay, I'm I'm a bit uh huh. In Christie, a patient that are bleeding. I mean, it's always a basement case basis. Um, on the whole, if if they're ill, they're going to be at high risk of thrombosis. So, um, unless several reasons not to the answer is yes. If they're so ill that their bed bound on do know highly inflamed and everything else, then yes, you're going to be giving trump prophylaxis. The only caveat to that is if they're bleeding, if they're very thrombocytopenia call, they've got some sort of bleeding diathesis. So you see either quite ill opathy and the blood work, or in, you know, the other parameters that would be giving you thought that they might bleed. You will be given them VD prophylaxis. Yes. So the default is yes. Unless the reasons not to um hum, you'll be fine. So we can, you know, very much change. Okay. Happy to do it