Transcription
Hello everyone. Can you hear me?
>> Yes, we can hear you.
>> Yeah. All right. So, I'm going to start the session. I hope everyone can see the screen. Is the screen visible to you as well?
>> Yes.
>> All right. So, welcome back to our hematology morphology sessions. Uh mainly for the part two candidates. Uh in the recent exam, many candidates struggle in the uh short cases. Um the best way to have morphology experience is to make your own own slide box uh or slide bank and your own microscope like this and uh practice with your own microscope. Here in the online session, I can only tell you how you can uh attempt the short cases or long cases. Uh but uh morphology is very practical in part two exam. Your eyes need to be need to be addicted to a microscope which you can take to the to the exam. This microscope I bought in 2023. It is an Omax microscope with a top camera and it is working. It cost just £350 at that time. Okay, so better to have your own personal microscope and your own slide bank and see the important slides for the for the exam exam purpose. Uh you are all senior hematology trainee or consultants. You will be seeing blood films, but the daily routine blood films are different from the from the exam blood film. You may You may You may not be seeing TPL cases every day. Uh so if you have any new diagnosis in your ward or in your hospital, ask your lab to make one one film for you extra and keep it in your slide box. And see them again and report them because it's not only the diagnosis which is important in part two exam. There are 10 marks question. Okay, you need to answer them and you need to pass them. Okay, and we have shown you the results previously as well. Many candidates failed by just 0.5 marks or one mark. Every mark matter in the part two exam. Okay.
In the back people in the in the gallery. So this is the first case. This is 19-year-old female. This is a 19-year-old female with a fever and fatigue. She has presented to the emergency department with a feeling unwell. The full blood count shows hemoglobin of 110, white cell count of 32, and a platelet count of 120. This is power 10 view that you are seeing at the moment.
>> So. Now I will go to power 50.
>> [clears throat]
>> All right. So you have seen multiple cells in this film. Would you clean the slide?
>> [clears throat]
>> I will need a volunteer to comment on this blood film, please. 18-year-old So, 19-year-old female with fever. What differentials would come to your mind? Um the people going for part two exam
>> So, obviously, it could be a viral infection. It could be um secondary to any underlying autoimmune process or hematological disorder or a bacterial infection as well. Uh I think these will be the normal, I would say, differentials.
>> Is what? So, if they ask you to report on on this blood film um how would you report it?
>> So, I would start with the most apparent finding. So, there's a marked leukocytosis uh with the some abnormally large uh mononuclear cells which are which have uh adequate cytoplasm, increased nuclear size, and and a nuclei is present as well. Uh and and and some of them look like a blast uh as well. Uh the red cells are slightly hypochromic um and there is anemia. There are occasional tear drops as well. There is mild thrombocytopenia.
>> Mhm. Your impression?
>> I think the um looking at the film as I'm I may have want to see a bit more, but I think my concern would be a hematological uh malignancy like BLL.
>> Okay. So, I'm a a first-year biomedical scientist. I have heard my senior saying that if there is any red cells scalloping by the lymphocytes or uh white cells, this is most likely reactive.
>> And reactive. So, what
>> Well, they some of them does look reactive, but some cells I think not in this field, but in some cells they were a bit concerning cell. They they can be reactive lymphocytes in a in a you know, in like a EBV infections and things like that. Uh um but I think I definitely was concerned about some cells that leads somewhere which uh and and given the general uh like patients being you know, more uh uh has a bicytopenia as well. So, that was another concern.
>> Okay.
>> I may be too
>> But you you have reported the blood film. You have commented on the cell line. Um Do you always give your impression as well? So your impression is?
>> My impression would be um a hematological malignancy like B L L. Yeah.
>> Okay. And what would you like to do next?
>> I would like to uh review patient and then I generally send for a flow to confirm the lineage.
>> Now your report is complete. You have commented on the cell line. You have given your impression. And then you have suggested as well. Okay. All right. So anyone who thinks that this is a reactive blood cells?
>> Yes, I think this is a reactive lymphocytosis.
>> How how you can say that this is reactive lymphocytosis because Danish is saying this is malignancy.
>> Um so that they they are um um pleomorphic and there there is a scalloping around the red cells. They're large. Um there is granulation. There is pro connect lymphocytes. Um so I in a in a context of a young a girl with fever um my impression was this is likely infectious mononucleosis like EBV uh viral infection most likely.
>> Do you think this phenomena uh these cells are sticking to one another. This is common in the viral infection.
>> Um
>> Yes, there is a red cells scalloping. But Mhm, go on.
>> Mhm. I'm not sure now. No, I I think um it's it's the fact that it's pleomorphic that makes me think that it might be more infective, but I agree these don't look like typical atypical um lymphocytes. Some of them do look concerning for blasts.
>> All right. So So my main point here is that people say scalloping is a feature of reactive lymphocytosis. This is wrong. Scalloping is not a feature of reactive lymphocytosis. Mhm. These are all monoblasts.
>> Mhm.
>> These are all monoblasts. And monoblasts have the feature of uh sticking to one another. When they are high in number, like if they are more than 100 um in a in a patient they usually lead to acquired von Willebrand syndrome. And the ghost chases effect. These are not a reactive lymphocytes. Reactive lymphocytes are very pleomorphic. They They They don't seem to be pleomorphic. And whenever the white cell count is high in number, they they will stick to nearby lymphocytes. Nearby I mean red cells. And it and you will feel that this patient has scalloping. So, red cells scalloping is not a feature of reactive lymphocytosis only. This is a misnomer. Okay, many people have reported acute lymphoblastic leukemia as reactive lymphocytosis. Why? Because they are scalloping. So, what? You you will not see these sticking effect in the reactive mono reactive lymphocytosis. These are all monoblasts. You can see these are promonocytes. A lot of a lot a lot of regulations here and here and they are sticking to one another.
>> Now it's obvious. Yeah.
>> Yeah, so They they can trick you in the exam like this. Yes, whenever there is a 19-year-old girl with a fever, we think that it will be most likely reactive lymphocytosis. But be vigilant. Leukemia is very common now in young population. Why? I don't know. But we have seen 20-years-old with very strange leukemia, 26-year-olds, a 19-year-old, 18-year-old. We have all the patients in our ward. They have leukemias. Okay. Okay, they do not seem to be a reactive lympho- lymphocytosis. These are all promonocytes and monoblasts. This patient had acute monoblastic leukemia. The next question would be um treatment. So, Danish, what treatment you will offer to this patient? This is a young patient, no other comorbidity is felt, unwell, came to the hospital, shows leukocytosis, and this is the blood film, and the flow shows um um AML in this patient.
>> So, um obviously there will be a uh I would start with the my um I think in the treatment we have to go through like everything. We will discuss the case in MDT. Uh then patient information, and then uh the treatment will be mostly uh uh for the her will be DA based uh um and then uh uh go will be depend on the uh with the flow markers uh and uh uh Yeah, I think uh it depends on the uh um another presence of the cytogenetics as well, whether we will consider uh uh a consolidation with transplant or um or not, but uh for now it will be a DA based treatment.
>> Um if this patient has complex cytogenetics, then you will go you will give DA or something else?
>> I think if she has complex cytogenetics, um as long as it's not uh you know, going towards the MDS type, uh um then I think DA based would be reasonable, but uh we may have to consolidate it with transplant. Uh but if.
>> Um
>> Standard cytogenetics as per the ELN criteria, they receive um and uh one of these cytogenetics are intermediate risk or complex karyotype or adverse risk, then we give them like either.
>> Or that side.
>> Yeah.
>> In the intermediate risk in the intermediate risk, we in the intermediate risk and the worst cytogenetic risk category, our aim is to transplant it them in the first uh CR. But if it is standard risk cytogenetics, um we do not transplant them.
>> Yes.
>> In the in the first CR.
>> cytarabine consolidation, yeah.
>> Yeah. So So they may ask you about the treatment. You should know about the treatment and as per the BSH 2022 guideline, the minimum investigations that you that you will send for every suspected AML patient. You know that what What investigation need to be sent for every AML patient suspected AML patient?
>> So we need to send as I say, we need to do um cytogenetics and then we need to do an NGS as well. Uh So at least to look for uh I think at least to look for uh NPM1, FLT3, ITD and TKD and uh uh Yeah, it should should we Can we just say NGS or do we have to write a more detail?
>> Four categories. One is NGS. Second one is karyotype. Third one is uh PCR or fish for inversion 16, 821, and KMT2A. And then the the fourth one is molecular for FLT3 ITD and TKD. This need to be sent on every suspected leukemia patient as per BSH 22 guideline. Let me just correct the volume.
Okay, so this is our second case, 8-year-old who is feeling unwell and came to the hospital for a review. This is the blood film, and the blood film shows hemoglobin of 90, white cell count of 60, platelet count of 90 as well. This is the power 10 overview of the blood film. Power 50 now.
>> [clears throat]
>> All right. Any initial thoughts?
>> The cells look like blasts. Mainly.
>> Mhm.
>> So, they are all moderate size with very high NC ratio and uh dense chromatin with occasional nucleoli.
>> Mhm.
>> And along with thrombocytopenia, anemia features.
>> Mhm.
>> [snorts]
>> Yeah.
>> There are no uh there are no normal uh myeloid or uh lymphoid series cells. Most of them are premature, blast-looking like looking like blast cells.
>> Mhm.
>> Uh Uh differential could be acute leukemia. Pretty short history.
>> Okay. What do you think? What type of leukemia this patient would have?
>> Uh likely ALL, maybe BL or TL.
>> We'll go for um high power possible. Okay. So, these these cells cytoplasm is free of granules. So, likely um ALL type. But, we always wait for the flow result to to confirm the lineage whether this is lymphoid or myeloid. Because in practice, sometime we think it is lymphoid and it comes out to be myeloid. Sometime comes out to be bi- biphenotypic and sometime a different thing. Okay. So, tell me the full report um for this blood film. You have commented on the cell line. You have given your impression that this is likely acute leukemia, which is okay. Suggestion what what you will advise on in your blood film report.
>> Uh we'll advise like to further confirmation by bone marrow aspiration and biopsy. Send for flow cytometry uh to uh immunophenotype this patient this cells then uh if it is leukemia so we'll send a lymphoid panel NGS. Oh. To risk stratify.
>> This patient will go home? Because the flow cytometry may take few hours. And bone marrow biopsy may not be done overnight.
>> We need to admit the patient we'll call the uh physician and inform the results that this patient need uh acute management, admission, hydration, uh tumor lysis, so workup.
>> We are suspecting this is acute leukemia. And this is 8-year-old child. Okay, so he would need admission and bone marrow biopsy under under anesthesia and all the workup for the leukemia. So for example this is B-ALL. Okay, I do not have the flow results to tell you whether this is B-ALL or T-ALL. How do you risk stratify ALL patients? Uh what features would make it higher risk and what features would make it standard risk?
>> Um mainly the white cell count if it is uh more than I think 35,000. And uh thrombocytopenia, age. If it is uh he's less than he's young young year young child so low risk. But uh his white cell count is more than 40,000 so he is stratify as high risk. And we need to wait for his uh NGS panel.
>> NGS panel may take few weeks. So, after reporting, this will be the question in exam. How do you risk stratify ALL or to be more precise, BALL patient? So, you have to tell about white cell count, translocations. Any Any translocation that is high risk?
>> Hypodiploidy.
>> Mhm.
>> Philadelphia.
>> Mhm.
>> Yeah, KMT2A KMT2A
>> Mhm.
>> Going Islam.
>> So, it starts with the presentation eight and white blood cell count and at younger ages carry better prognosis. This patient white cells count was 62. And then when we do the bone marrow investigations, we need to look for the fish and karyotyping and uh further NGS panel. We particularly fish for BCR-ABL. And we fish for MLL and PBX as well. So, this is uh translocation that we do uh at the beginning to indicate poor risk or poor prognosis. And then the karyotyping it will take likely 3 to 4 weeks or longer. Uh but we're looking for hypodiploidy. We're looking for um deletion 17 and 7 I believe, yes. And we're looking for complex complex karyotyping as well. Like this is all um carry high risk prognostic features.
>> And the standard risk?
>> So, any um things apart from that this is carry the standard risk, which is um younger age, uh white cells less than 13 B L L in particular. Um and any other um features not um in classified high risk. And um in children's house location 12 21. And hyperdiploid we do as far as I remember.
>> Good work. And the next question will be treatment. What treatment can you offer to this child?
>> Um
>> You call 11.
>> so, yeah.
>> Sorry? You call 11? Currently you call 11 is not used in UK. Is it the
>> UK protocol as the
>> Is that
>> And all together protocol I think UK has used.
>> It's all together protocol in UK currently. UK 14 is also for younger population, not for 8-year-old child. Okay. The all together protocol is very complex and long. Um We We have shared the infographic with you uh the about the algorithm of all together protocol. If you can memorize that for the wire purpose in part two exam only. But you are not expected to write them in the morphology short or long cases, but they can ask you which patient would be in standard risk category of all together protocol, which patient would be in intermediate low risk, intermediate high risk. Um, these things you should know. And they may ask you about blinatumomab up front or
This is another 30-year-old patient who is feeling unwell. His hemoglobin is 110. White cell count is 14. And platelet count is 110 as well. This is power 10. It's slightly old blood film, so you may not see things clearly on that. I will try.
>> This is power 10. And we will go to power 15 now. Wait. Do we have any volunteer from those appearing in part two? Pick up. There is any volunteer?
>> I can have a go. Although I'm not sure, but let's see.
>> Yes, go on. What What have you seen in the blood film? You report the blood film.
>> So, they are uh medium to large uh polymorphic cells. Um in particular, some of them that are hypergranular. Um not all of them have got nucleoli. Um Yeah, some of them Uh most of them they are hypergranular. It's concerning for promyelocytes for me. Um and then looking on the uh platelets Although that you said the platelets 110, this is film looks thrombocytopenic for me. Um some anisocytosis Uh sorry, I can't see anything anymore.
>> Yes. Yes. Making the
>> Yeah, this is a bit of um hypergranular, high NC ratio. Um concerning for promyelocytes um for me. As I said, this film looks uh thrombocytopenic. I'm not sure how platelets 110. Uh there was cut um few uh red cell fragments as well. Um the um What else did I mention? So, white cells, platelets thrombocytopenic for me. Um um nucleated red blood cells as well, few hyperchromasia, red cell anisocytosis. Um I think this um blood film is concerning for um possible acute leukemia, likely APML uh in view of the uh promyelocytes suspected blast. Um this patient needs to be admitted to hospital urgently. Send the uh EDTA sample for flow cytometry and check the existing or send for blood count or not for blood count, sorry. U&Es, LFTs and globis screen for this patient immediately and we need to admit the patient urgently and treat for the emergencies as well. That's how I think about it. Oh, yes. Flow cytometry and fish for PML rather if I suspect it which is I did and admit the patient urgently as I said what we're going to do for them. There's some biology here as well. Um
>> Sorry, what you will do?
>> I said that Sorry, yeah. So and then if I suspect patient which is I did for APML, I think I'll start at her.
>> Okay. Yes.
>> So you said the white cells is 14 so it's more than 10 so yeah, I'll start at her. Well, yeah. I think this is a short case. I wouldn't be able to say more than that. If it's long case, I'll be talking more than that.
>> Mhm. So APML highest APML which needs admission and um starting chemotherapy so just starting at her as soon as possible. And you will send when when you mention the clotting screen always say fibrinogen. And then because in some hospital fibrinogen fibrinogen is a part of the coagulation screen. In other hospital, it is derived fibrinogen. And we are interested in closed fibrinogen.
>> Yeah, correct.
>> because the patient may have bruising or bleeding so you may be worried for the DIC in this patient. Right. So you have started the patient on at her. What would you m- monitor for?
>> So this white cells is 10 first of all we need to Sorry, 14 is that So, this is high risk. So, usually it will be either starting the patient on additional site reductions such as either rubicin and some other hospital hydroxycarbamide as well will be concerned about the differentiation syndrome. So, we will be assessing the patient as a baseline for risk of differentiation syndrome and if it's high it's in this case likely depends how you're going to treat sometimes we do start prophylactic steroids dexamethasone 10 mg BD sometimes we don't it depends on other biochemical markers how the patient is doing what's the baseline chest x-ray what's the baseline fluid status but if we suspect I think it's just easy to give the patient dexamethasone 10 mg and BD and then until we achieve safe site reduction and then we can stop it accordingly. And then yeah, if if you ask me about the whole things that this is will depends on from A to Z for patient counseling but it will be like emergency so MDT wouldn't be like an immediate things but many MDT will do that. Counsel the patient and consent them for chemotherapy and the risk documented the high risk complication of this treatment including differentiation syndrome and catastrophic bleeding thrombotic and death as well. And then we need to TLS risk assessments and starting TLS risk management such as rehydration in this patient but we need to be careful because this patient will be at risk of differentiation syndrome so fluid balance assessments and prescribing the fluids accordingly will be the optimum management for this patient.
>> Do you expect any electrolytes abnormality with atra?
>> Um yes, I we
>> ECG abnormality?
>> Yes, we do have the baselines of magnesium level phosphate, calcium and all of them and QT prolongation could happen. So, we need to be careful about it and yeah, do all our physical assessments from A to Z. As I said, I'll just give you this because this is short case.
>> [laughter]
>> If If it is a short case, they recently they have asked about counseling section in the short cases as well. So, we can ask you anything.
>> Yeah.
>> And um if they ask you about monitoring on arsenic or or the um atra, you need to mention about differentiation syndrome and the electrolytes and QT interval.
>> Sure.
>> What is differentiation syndrome and how do you treat it?
>> So, differentiation syndrome is a kind of hyper proliferation um of the promyelocytic cell and uh including the third space fluid accumulation. Um this is um an emergency which is good could be fatal. Um we do treat it the starting by the uh assessment pre-starting the treatment which is uh in a high risk cases we do start prophylactic uh dexamethasone. If the patient has happened to have suspected um uh differentiation syndrome, it depends on the assessment of the risk um and the um uh fatality of this condition. If it is really patient is really unwell, in that case we stop treatment and we do treat the differentiation syndrome which is including ABCs, and we usually take this patient to high dependence unit ITU to be treated over there. And if sometimes with MDT decision if this is intermediate risk differentiation syndrome patient is not completely unwell, and then we do continue treatment bearing in mind there is other complications such as DIC, bleeding and thrombotic complication of this patient. Patients sometimes get a fluid overload as well, which is we know this is the main stay of the presentation, which is acute respiratory distress syndrome, fluid overload in the lungs and peripheries and third space accumulation. So, we do use diuretics and strict fluid balance for these patients. Um Uh yeah, and continue dexamethasone 10 mg BD until we achieve um resolution.
>> Did you stop Ativan?
>> Yes, we did. As I said, if it's high risk, yes, we stop treatment and we treat for that. Sometimes in a few occasions decision will be if it's mild to moderate severity, and then we can continue on that. But the main stay if it's high risk patient unwell, we just completely stop it.
>> Diuretics?
>> Yes, mentioned already.
>> Daily weights?
>> Yes, mentioned already for strict fluid balance and fluid management.
>> All right. All good. Remember, it is a 9-minute 9-minute short question in exam. So, you have to be quick in writing um in writing your stuff in bullet points. Somebody has CNS prophylaxis. Can you elaborate about CNS prophylaxis?
>> Is this for me? I did not mention CNS
>> In the chat, somebody mentioned CNS prophylaxis.
>> We don't usually give CNS prophylaxis by default. Um this is it will be on case by case. Um but it's unlikely we give prophylaxis. You know, IT chemotherapy compared to other leukemias for example ALL others. Um if um the patient has had LP at the time of the diagnosis or any other reasons not hematological reasons and it deemed to be traumatic. And then and MDT discussions about investigations, imaging, and considering of that, but this is not very common. Um but if this is has happened, we do assess for it separately.
>> Right, correct. Thank you.
>> There's something called tumor cerebri which you get as a side effect of the ultra as well. Um I don't know if this is what what the person who mentioned, but it it isn't it it's a it's a side effect. It's tumor cerebri. You see that.
>> Yes, pseudo tumor cerebri is a side effect, but we do not give any prophylaxis as per your protocol.
>> Yeah.
>> And if the patient has developed DIC, then we keep the platelet count above 20. Above 50 I mean.
>> That's right. This is when we reduce ultra as well. Sometimes we we reduce.
Is that another patient? who has traveled back to UK after having a trip in Brazil and Congo. Now he's feeling unwell. So, this is his power 10 blood film. So, whenever you you read a scenario in the exam and it says traveling or you have seen full blood count and it shows high eosinophil count, you should start from power four. Okay. Do not uh see your slide at power 10. Start from power four because Niquas slides, they are full of parasites. Okay. Even if you see naked with naked eye, you may see parasite in Niquas Niquas slides. But in exam slides, the whole slide contain only one parasite. Okay. In our exam, one of the uh a few candidates' slide did not contain any parasite at all. So, that MCQ was removed then from exam. So, whenever you see things about travel and or high eosinophil count, then start from power four. Let's see what you see on high power here. Patient is feeling unwell. And he is not responding to antibiotics.
>> It's a bit blurry. Yeah, it's better now, I think.
>> Yeah, this is a parasite blood film, which is by default, I think, blurry. We'll go to power 100 to show you these things. Can you see these things? It will take time to focus. Can you see this?
>> Oh, yes.
>> Yes, it's obvious.
>> We can see that clear.
>> C-shape um trypanosomes. So, this they can appear in exam as well as they have appeared before. And um your lab your lab would contain um parasite blood films quite a lot. Um among the among the worms Loa loa is quite common in UK. And they are the one which usually appear in the exam. So you should see them must and before going to your part two exam. This is another parasite. I'm trying to focus it. It's C-shaped. So what is Trypanosoma known for? What it cause? Anyone?
>> Sleeping sickness.
>> Sleeping sickness.
>> Sleeping sickness.
>> Peri-orbital swelling.
>> Filariasis and visceral leishmaniasis I think.
>> Mhm. So Trypanosoma causes
>> Hematuria.
>> Um No.
>> Chagas disease.
>> Hematuria is caused by Schistosomiasis. And orbital swelling would be caused by Loiasis, the Loa loa condition or Loiasis something like that. Trypanosoma is known for two diseases. Um I think the Trypanosoma cruzi causes Chagas disease. Um and Trypanosoma brucei is known for
>> Confusion.
>> neurological involvement
>> sleeping sickness, African sleeping sickness
>> This is a tsetse fly which give the um infection.
>> Yes. So, this slide contain a bit more Trypanosoma parasites. But, I'm sure if you go to your labs and ask the biomedical scientist regarding the parasite box it will contain Trypanosoma. One thing else that you need to know for the exam purpose is the names of the drugs that is used in the treatment of Trypanosoma. We usually do not deal with Trypanosoma as hematologist but for exam purpose we should know the the names of the drugs that is used. The only name which I remember is the pentamidine and nifurtimox. There are many new drugs now. But, you can Google it and open CDC website. They have few names for the parasites medication. This is also and how to differentiate between Trypanosoma cruzi and brucei it is out of the scope of our exam. So, do not worry about that.
>> So this this section contain more trypanosomes. All right, so do not miss um the parasite films. They are equally important. One parasite every exam.
And the last blood film. This is 30-year-old female. And this is a GP blood film as well. Okay. And after this blood film you can go to the beach. Um it's very sunny today. So this is power 10. You can see the status of white cell count here. It's a very white very high white cell count. You can see that. All right.
>> So now this is the blood film of a 30-year-old female sent to you by GP and the first year biomedical scientist has mentioned in the comments leukocytosis likely reactive need to ask patient about infection. And now the blood film has come to you to be reported and countersigned. Okay. So if I ask Hanim Anum can you hear us, Hanim?
>> Yes, I can.
>> Yeah. So you are sitting for part two exam.
>> Uh yes.
>> Okay. And you have been asked to report this blood film. So please report this blood film for us.
>> So this peripheral blood film shows hyperleukocytosis. Predominantly immature granulocytes are seen including promyelocytes, myelocytes, and metamyelocytes. Also mature neutrophils are also visible. Uh I can also see eosinophils. Occasional blast cells are also seen. Uh Red blood cells are slightly hypochromic. Platelet counts are looks normal to me on peripheral blood film. Uh my initial impression is myeloproliferative disorder, likely chronic myeloid leukemia in chronic phase.
>> What next? So, you have reported the blood film. You have given your impression. And the third part of the blood film reporting is to do what? We have just discussed in one of the case.
>> Is this a GP blood film?
>> Yes.
>> Okay, then then I will call the patient and ask about the symptoms and uh
>> What is this? You didn't mention the blood
>> Sorry. Uh-huh, yes, I didn't mention. These are basophils.
>> Okay.
>> Okay, so then I will call the patient and will let him know that I will ask his GP to refer back him to us and followed by the further investigation.
>> Do not change the gender of the patient, please, in exam.
>> All right.
>> I mentioned this is a 30 This is a 30-year-old female.
>> 30-year-old female.
>> You mentioned I will call him.
>> You're right.
>> Okay.
>> Okay, so I will call her and let her know that I will ask her GP to um let her refer back to us followed by a further investigations in the outpatient clinic starting settings including uh bone marrow um aspirate and trephine biopsy, bone marrow cytogenetics, and uh BCR-ABL transcript BCR-ABL analysis by quantitative analysis by PCR.
>> I will not ask her GP to refer this patient to us. If I see a a patient blood film with a white cell count of more 100 more than 350, I will ask her to come to the hospital, please.
>> All right.
>> Okay. The GP may not receive our referral or he may send a normal normal referral to the patient for the patient and we will pick that up after a month. By that time, her spleen would have burst. Yeah. So, you need to send in in the report section in the end you have to mention this is most likely MPN. We will send um peripheral blood for BCR-ABL for this patient to confirm the diagnosis. And I will contact the patient myself to to find out how is she if she's symptomatic or not. This patient need to come into the hospital for further assessment. Okay. All right. What further investigation would you like to do in this patient?
>> Uh starting from bone marrow biopsy. Bone marrow cytogenetics. A quan- quantitative BCR-ABL by PCR.
>> Any other test? You're thinking this is a myeloproliferative disease and white cell count is very high.
>> Yes.
>> Okay, you would like to reduce the white cell count, all right?
>> Right. So, I can also check the uric acid and LDH.
>> So, in suspected CML patient, we do Q risk scoring. Which would need um the patient HBA1C baseline lipid profile. This is a young female. Pregnancy testing would be done. Bone marrow biopsy you have done already. The routine blood test which include organ function test as well. Yeah, this white cell count is very high. You You would like to give this patient hydroxycarbamide. So, baseline TLS bloods you would like to do. Okay. What What treatment can you offer this patient if this is BCR-ABL positive?
>> Uh so, I will start with the first-line tyrosine kinase inhibitor like imatinib 400 mg once daily.
>> Okay. You're going for part two exam. When they ask you what treatment you will give to this young female, you will write I will calculate first the ELTS score and I will look for the bone marrow report to find out if there is any uh major root abnormalities present or not. I would find out if this patient would like to get pregnant in the future in the near future or not. Because if the ELTS score is high or if this patient has major root abnormalities, okay, which are mostly trisomies or 17p, etc., then you can give them second generation um uh TKIs, which is either dasatinib or nilotinib. And high ELTS score in young females uh having um pregnancy plans in near future, we do not give them imatinib. Because dasatinib usually bring them into remission quickly. All right?
>> All right.
>> Okay. So, ELTS scoring is important here. Previously, the people used to use Sokal scoring or Euro score scoring. Now, because of the TKI era, we use ELTS scoring. Okay. So, your Your answer should be like this for the examiner. CML has appeared multiple times in in viva and the blood film appears in um in the short cases as well. The blood film usually have very few number of basophils just to confuse the candidate whether this is reactive blood film or CML blood film. Obviously, if someone has 400 white cell count like this blood film, it cannot be a reactive blood film. Reactive blood film is mostly up to a white cell count will be up to 50 or 60. Usually, they do not go high higher than that. Rarely, you will see any reactive blood film where white cell count is is 100. Or if your patient is continuously using GCSF and you have not stopped that, their white cell count may reach 100. Otherwise, a patient with 350 or 400 or 450 white cell count like this, like this picture. It would be an MPN case, most likely. For example, this patient has leukocytosis like this. And she has epistaxis as well. What would have you done differently in the management of the patient? This patient is in ED. She's bleeding from nose. You have done blood test and seen blood film. Uh white cell count shows uh white cell counts are 400. And this is the blood film of the patient. What you have done differently?
>> Uh I think she has developed hyperviscosity syndrome.
>> Mhm.
>> Um So, we need to check plasma osmolality and and if it's high, then uh we should go for a leukapheresis.
>> Mhm. Yes. Take blood sample for von Willebrand screen and discuss the case with the apheresis unit because apheresis unit is not present in all the hospitals. Okay. And uh uh request for leukapheresis in this patient. The patient may need one session of leukapheresis or two sessions of leukapheresis. And uh the platelet count will go down. So, I mean, the white cell count will go down. So, just blood for coagulation screen including fibrinogen and the acquired von Willebrand screen. And then look for the resist for the patient. And then you will give the remaining treatment as we discussed earlier. Right. Um any question? For the last case. Neither you can see the dosing of amantadine and naloxone in children in the SPC. Uh I do not remember them. Sorry. Right. Any question? Shout out. If not uh I'm going to
>> another question, sorry.
>> Yes, go on, please. Karin, you should stay online, okay? After everyone goes.
>> Uh sure.
>> Mhm.