Transcription
Let's start today. We have four blood FMS and one aspirate. Yeah, so this is a 48-year-old male who was referred to us to hematology by a GP because of persistent and worsening anemia and thrombocytopenia. On examination, he mentioned that, um, he mentioned that he has some abdominal discomfort as well. And on examination, he has plum Al 17 cm. His hemoglobin is 60 and his platelet count is 90. White cell count is 7. Anyone wants to volunteer, comment on the blood? This is power. Okay, any volunteer? No one today. Yeah, could, could you focus in a bit more? Yeah, I will move to power 50 oil. Sign HP cell. Um, this is a, uh, AR. So this is not a, but a significant cell is this one, this one, and this one. We will see some more, uh, sections as well. Why go time and some features here? This one, this drop, kill site, this one, toxic granulation, maybe of the neutri has granulation andc here. Drop again. All this is going on now, especially here as well. Can we see a couple of neutrophils, please? Neutr. Okay, yes, please. Yeah, this is a bit of bend for. Yeah, Ben, the a normal with Dr. What was the HP 60? Constitutional symptoms as well, and SPL megal? Yes, patient has 17 cm of splin. Okay, yeah. And sorry, plastic anyway. Yes, I agree with that. This is liid blastic blood. You statocytes, few statocytes as well. Yes. What about the red cells? Hosis. The blood fin contain red cell and Asos phytosis with few polychromatic mcroy. There are Deyes or here, drop cell, you can say that. Multiple NBCS. I'd expect a few more to be multiple, multiple F. Yeah, MF. Sorry, it should be few more te adoption there. Yeah, this one is a te drop. Yeah, this is going to be a te drop soon. And we have seen quite a lot, um, in this patient. Only one thing which I was searching was for any Howell Jolly body because the patient has clean. Yeah, or maybe this one, but not very clear. So this patient has both polychromatic macroy. We have seen few pyes as well. There are, uh, multiple te drops in this patient. Liid blastic fure. This Blood film is most likely consistent with MF. And MF does not come alone with the hemolysis or spherocytes or polychromatic macroy. So this patient has most likely dual diagnosis. One is likely MF because we have to confirm that on Bonner biopsy. That's why we are saying likely MF. Yeah, because of the Luc blastic picture and here dropping and there is some hemolysis going on, which is evident by the presence of spiroides, poly chromatic macroy. So what would be the recommendation? Like in your report, you have to write some recommendations. This patient needs bone marrow biopsy to confirm diagnosis of myofibrosis and hematinics to see for any B12 or FID deficiency. So this patient on biopsy had Milo fibrosis and had low folic folic acid. So this was a case in the recent, uh, in the short case of hematology morphology. So reporting the blood Fame. The blood F contain contains multiple nrbcs and tear drops along with the presence of spheroides, polychromatic necroid. The platelets, the pl count is low. No colum seen. White cell shows, um, multiple band forms of Nutri, toxic granulation in the neuts and some neuts without granulation. The picture is most likely lucar blastic, which is consistent with myofibrosis and, uh, some ongoing hemolysis. The patient needs Bob biopsy to confirm the diagnosis of myofibrosis and hematinics to look for any henic. This patient hemoglobin was 60, pled count was low, and the blood frame does not show any Bloss. We don't know about the vulner biopsy result yet. White cell count was 11. And let's say he's transfusion dependent as well. Anyone knows which dips category he would be? Dips low, dips intermediate one, dips intermediate two, or D high? So he will be dips intermediate category, uh, two. It is a high dip score. And, uh, in intermediate two dip score, we first address the spag because this patient has 17 cm of splin and he is in discomfort because of the splin. So he would need first a treatment with jet2 inhibitor, which is rotin. But if the pled car drops to less than 50, then we cannot give him Rox solutin. Then there is a new drug called Moten to give to this patient. According to the nice guidelines and V guidelines, m is now nice approved. You can give it to your patient if their pled count is below 50. But if the pled count is above 50, you can try roxil first as well. For the anemia, because a patient is now symptomatic, you have to give him blood transfusion. But, uh, you need to check the EPO level of the patient as well. If EPO level or erotin level is low, try, um, erotin stimulating agent EO first. If the patient respond to that, it's okay. If no response byol and check the response in, uh, few months time. If he respond to dinol, it is okay. If no respond to dinol, then you have to go to transfusion program. And then because it was a high risk in, uh, High, I mean the dip score is intermediate to such patients. We usually discuss in transplant MDT. If, if the patient has no comorbidities, patient agrees for transplant as well, and Transplant MDT also agrees, uh, for transplanting him, then such patient fate is allogenic Bal transplant. Is there any question that you want to ask about mof fibrosis? The new thing in manop fibrosis is that mottin has been nice approved recently. You can use it as a first line. You can use it as a second line. The price of mtin is the same as the Rox. It, the the side effects up till now, they are very less. They are only gastrointestinal side effect. Patient develop diarrhea or woman. Up till now, there is no other serious side effect. In case of Rox solip, you need to Wi it down. You cannot stop it, uh, at once, otherwise the patient will develop, uh, inflammatory response. You need to we it down under cover of steroid or the RO. We do not use ptin in UK because of the, uh, risk associated with, uh, pamine deficiency and luil opathy. So most of the Consultants do not use ptin here. Yes, go on. Dr. Can you please explain what is the role ofip in increasing the HP? Does it reverse the fibrosis or what? So the rotin is for, according to the conferred trial, for symptom control only. It will help in reduce in the spleno. For the anemia, we will check the arrin level of the patient. If it is low, you start the patient on EO. If the patient respond to that, it's okay. If the patient does not respond to that, then you transfer to denol. If the patient respond to denol, that is fine. If no response, then you have to think about other options, which is mottin these days. But rotin does not in hemoglobin level. It reduces the spleen size only. And according to comfort trial, it is for symptom control. Okay, hopefully you guys would know what were the cases in part two exam philosophy. This was one of the sorry, Amir, can I ask whether we are required to know about the scoring system and how to calculate them? No, but you should know the names of the score. Yeah, okay. Uh, because it is very difficult to remember the components, components of di score. It's very difficult. And there are multiple scores for monop fibrosis, dips score, U mips score, mtss. The transplant, those patients who are going for transplant, the transplant MDT will calculate dips score, the mtss score for the patient. But in routinely, in routine, the majority, the most of the score which is used in practice is dips score for the. Okay, thanks. So when you see any clinic letter of a myofibrosis patient, on the top, you will always find what is the DI score of this patient. That he is a low, low risk category, intermediate one category, intermediate two category, or high category. In the recent exam last week, there were a bit, uh, a few changes in the short cases as well, which were they asked about the management of a disease in a short case, which is very unusual. So people has a lot of time management problem in this exam. This is a second case. This patient again, this is a 50-year-old patient admitted under respiratory team because of the pl, plural effusion. When they did the, uh, Bloods for the patient, the white cell count was high. The white cell count, you can see from the picture, it is more than 80. So I would like any of the EMS or, um, lab scientist or lab fellow comment on the blood spin. What they think? Well, there m Lucas Tois there. Yeah, can't make the cells out. Yes, so this is power 10, just overview of this slide. Yeah, high, very high. And now I will move to power 50 to make them more clear. Bling on the olympos sites there. Yeah, that stands out. Lot of BL P something like that. Sorry, which dis a PLL? Yeah, as well. BPL or TP. T. T. What was the pl come? PL is 97. Okay, since is reduced. Yeah, a lot of bling. Yeah, right. So you have referred this Blood fin to the clinical hematologist. Any clinical hematologist want to report this pin for the purpose of o exam? Any clinical hematologist? What happened today? Everyone is, yeah. So no second, um, so, um, the blood film is showing a population of mononuclear cells with increased, um, NC ratio. The nucle, there is clear, um, nucleis in most of the cells and the chromatine c youit not focused and put some oil on it. Yes, this is power 100. Um, the the nuclear chroma appears a bit clumpy. I think I, I'm not clear whether does that's that's, um, like this is mature cell. So this is mature. Okay. On the cytoplasm is showing, um, um, cytoplasmic pling, a granular, a granular cytoplasm is someing. Okay, that's it. Um, so features suggestive of, um, LPD, Lop portive disorder. Further assessment, uh, and investigations are needed. So I will do bomber biopsy and send flowetry. Okay, so the, the blood film contains leucocytosis. The vo cells are small and mature with, with multiple cop plasmic blooding. The nutrifil, the the plated count is low and no plated plum seen. The right cell morphology is normal. Most likely consistent with proliferative disorder, likely PPP due to the presence of cytoplasmic ling. We need to send the flow to the lab for urgent flowetry to confirm the N. Okay, what flu do you expect in this patient? So you said it is TPP, so T Cell markers will be positive in this patient. But can you tell me two markers only which are specific and strongly positive in DPL? T Cell markers with strongly positive CD7 and CD52. If positive and this confirm that this flowetry belongs to TPL. Do you know what is the mutation or translocation in TPL? Inversion 14 and what mutations is involved? MTCP1. Okay, this is the mutation that is involved in TPL. Remember these things for both part one, part two exam. It can be an MCQ or it can be a one or two mark question in short case. All right. So this patient has a lot of PR diffusion. He has thrombocytopenia as well, because of which he is now symptomatic. He has shortness of breath, productive cuff requiring oxygen, and, uh, there is spontaneous bruising in him as well because he has throy. What will be the first line of treatment that you would likely to suggest to this p? Is it tell to zoom up? This is half answer. Alm are followed by allogenic stem cell transplant. Alen stem are transplant and in mission. This is the complete. You will get a full marks if you mention like that. Alm to up because PA is symptomatic, followed by allogenic stem cell transplant and first. Okay, right. So the third case is about a again 50-year-old lady who has a previous diagnosis of MDS with EXs plus. She is admitted because of the lower respiratory track infection requiring oxy. And the white cell count from the analyzer is 42. Usually his lost, his white sell count will be around 11 or 12 as a baseline, but today when she is admitted, our white cell count has jumped to, uh, 42. This is power two, uh, 10, just an overview of the blood thing. To is lot clumping. It's transforming, probably. Yeah, this is the, the first thing which comes into the mind of every person, I mean, from hematology, that this patient is transforming to AML. But let's see whether he is transforming to AML or she. Right. So the lab scientist or your lab colleague has mention this patient is likely transforming to AML. Film, film referred urgently to hematology regist for reporting or for expert opinion. So I need a clinical hematologist to report on this. There are nine people in the meeting and there is no clinical hematologist. Hello. Yes, hello. Yeah, uh, I'm Abdul. I think the grostic series shows lot of displac and there are lot of, probably more monoblast and prono sites. So some of I think I saw some or or roads also in some of the cytoplasm. So on this f nrbc, n RBC, myo series, I can say that either this is mety or a big band from Milo with Gran. This plastic band from Milo with gran metam and wec here. B from metam. Yeah, I am see many blasts there because the patient has previous history of MDS with excess blood. Okay, this one has fi but a lot of granes as well. Maybe Pro series, but not full-fledged blust. But we will expect blust in this patient because patient has previous diagnosis of MDS with. Now your BMS is with you in the lab and he wants you to teach him or tell him is this AML or not? What would you say to the your left Le? I think we'll have to do a differential count, differential blast, blast. But from the picture, what do you suspect whether this is converted to AML or not? Convinced. Yeah, what do you think is the reason of this of my Lord series and ANC and why the analyzer says that the white cell count is 42? It's probably counting the nuclear rbcs as lymphocytes. Yes, the RBC has been counted as white cell count. This patient has a lot of nrbc in the background. There are lot of, there is a myoid peak. You can see B forms, you can see myoides, metam, myoid. Some analyzers, some analyzers don't count the nuclear RBCs, although the newer ones sometimes do count them. But this is where the film comes in. You'd have to do a manual differential. We have, we have. Yes, I do. They they pi up N RBC as sometime, but they don't give you. Well, depends if the analyz has been set to do nuclear RBC. Ours haven't. Yeah, we'd have to do a manual differential to correct the white cell count. Yeah, when the differential came out, it says that 39% of the word blood cell is NB all and the reason for this a lot of RBC and myosite peak, myoid peak is because the patient was, uh, septic. The patient has low respiratory infection and requiring oxygen because of the sees the bone marrow is under stress and that's why you are seeing a lot of, uh, nrbc in the blood F. Yeah, there is no transformation in this patient. There is a septic response in this patient. That's why there is a lot of nrbc and myoid peak in the blood. So we didn't marrow this patient looking at the, uh, full blood count report. We think that this patient has gone into AML. We should marrow it. But when we see the blood fil, when we saw the blood fil, the blood fil was different than AML. So he reported the blood film contains multiple or loads of nrbc with myoid PE containing nutrifil, band forms, myoides, metaloides, and blast. L count is low. No clums seen. Most likely, um, a bone marrow response to, uh, to the infection. The patient need, uh, blood cultures and treatment of the underlying infection or and treatment of the underlying cause of bone marrow, under pressure or stress. Treat patient needs treatment of stress bone marrow. Substance was a case in the recent, uh, exam. Right? Yeah, you send out, uh, a manual differential count every few months. So this would probably be a good one to do. Yeah, I agree. So the white cell count here is 42. Can it, can this be a CM? Oh, not. I can't see any P fails. The fails there. Yeah, that differentiates from the from the CML because CML has myoid peak as well, but here we cannot see any basill andoil. The next question is, can this be a chronic nutrific leukemia? I don't think because of the presence of displasia. Yes, so chronic noic leukemia contain, according to WH 2022 guidelines, contain a mature neutrophils. The white cell count should be above 25 and 80% of these, uh, White should be mature neutr. Then you complete the criteria for a diagnosis of CNL. And CNL means chronic nutrific leukemia. And chronic neic leukemia has a specific mutation as well. CF3. [Music] Um, CF3. Which one? I forget the the digit, the four digit. CF3 S1 or CS F31 something like that. Check it in the Google or the book. So this is another 50-year-old patient referred to the hematology Department from the GP because of the yilia since 2 years. The GP has investigated the patient thoroughly for a reactive cause, but he has not found any reason for us ail. So he has referred the case to you for further consideration. The patient is completely as this. We travel history, no travel history. The GP has investigated the patient for a secondary cause. All the secondary causes or reactive causes ofilia has been ruled out by GP. That's why he has referred the case to you for consideration of clonal causes ofilia. This is power 10 and we will go to our 50 now. So the kind ofs ignore these kind of sign. These are not trial cells. They are just the ad effect because of the old sample. You can see the US here. They are regulated and larger than the normal degranulated. That could be because it's an old sample too. The USS are quite L and they are regulated as well and they are degranulated and some of them are like first bursted as well. So what do you think whether these USS are this plastic or not? Yeah, nucleus does look abnormal. Some of them. Yes, their nucleus is abnormal and they are vacated as well. Normally, the euil does not contain vacation. When they are vacated, it means there is something going on. Along with these n, they are they contain a lot of V. Yeah, okay. Anyone to report this Blood thing? The clinical hematologists are sleeping today or what is the reason? There are many now Shak Nting Hospital, Saha Ali, Kings Hospital, think I guess apart from this dis plastic is fils, I can't see any significant feature. There is no features to suggest an MPN or LBD as cause of the, uh, colonal eilia. Likely first. No, no block as well. Report the blood. There is an increase aeno fils with this plastic features, evacuation, an abnormal nuclear separation. The blood, the blood film contain eilia. Yeah, eils are abnormally large with multiple vations and this plastic nuclei. Red cell. Yes, go about neutr. Does he have neutropenia? There is no neutr. We haven't seen any neutr yet. So he had, he so he has, uh, neutr. Yes, okay. What's the HP? HP is 1010, sorry, 110. Okay, this is okay. So you have mentioned that the, the blood Fame contain leucocytosis and, uh, the blood has ailia. Unops are abdominally large, multiple nucleations and abnomal nucleo present as well. Uh, there is no plated clums and plated look normal. No red cell changes in the blood F. Most likely consistent with, uh, eilia. The GP has excluded reactive CA. We need to, um, investigate this Pati or chronal causes ofilia. Right. And for the chronal causes, you will suggest some test which I will ask. But first of all, um, what are the causes of clonal eilia? Do you know any cause of clonia? PD GFR a, PDGFR r b and FGFR1 mutation. This is one category. The other two ceg, hyper eosinophilic syndrome. Okay. And what is the third one? Chronic is inic Leukemia. Chronic is in. There are three categories of clonal. Um, I was about to ask you about the molecular targets in yilia clonal eilia, but you have mentioned ppgf a, FB, FGFR1. They are correct. There can be J2, ATV6, 3 as well. Okay. So this patient has a cilia since two years, 20 since 2022. No reactive cause. The, USFS are dysplastic. No molecular target are found in this patient. Means there is no PDGF a, PDGF r b or anything like that. Patient is asymptomatic. What diagnosis you will give to this patient? Synd. So you said adipic hyperopic syndrome. If you write this in the exam, I will give you zero marks because I told you this patient is asymptomatic. The word syndrome will come only if there is a symptom in the patient and syndrome, Dr. Means you will start treatment for this patient as well. While inic hilia alone means there is no symptoms and you will watch and wait for the patient only. Let's say this patient is diagnosed with eipic hyperic syndrome. Now patient has develop symptoms. What is the first line treatment for idopathic hyper anilic syndrome? Sorry, according to tissue damage. If, no, no, what is the treatment? Treatment of tic hyperic syndrome? What you will give to this? Steroids. Yeah. And he did not respond to steroid in 3 weeks time. What is the second line? Steroids. He did not respond to steroid in three weeks time. Low do IM 100 milligram. It's not low dose. It's a normal dose. 400 milligram. You will try low do only if there is pdgfr a rearrangement in the PTI. Right? Okay. So the last case is a aspirate. E. So this is power four. I don't know why there is so much like on. So this is, uh, again, a 40-year-old young man who is admitted from emergency department under hematology because of anemia, high white cell count, low plated count, and pain in the right groin. On examination, the right groin has a big Mass. Because of the anemia, thopenia, this patient was mared to see for the underlying theology. So this marrow has particle which is shown here under power four. So it, it is a particulate. Patient is 40 years of age and the aspirate is very, very cellular. Now power 10. Do you have any differential in mind for this patient or not? Patient has constitutional symptoms, anemia, thrombocytopenia, and a big mass in the right grin. It could be a lymphoma. Yeah, it is a lymphoma because he has a m. What type of you? DLBCL. DLBCL usually comes with widespread lopy and this patient has only a m concentrated in the groin area. Right? If I go to Power 50, you will recognize the diagnosis. I was just asking whether you have any, uh, whether you have anything in mind from the scenario or not. Is it lymphoblastic lymphoma? Lymphoblastic lymphoma. What is lymphoblastic lymphoma? I said so because of the hand mirror like morphology. Maybe I may be wrong. What else do you see? Smart said, what else? Again, if the first candidate mention ly blastic lyoma, l blastic ly come with is usually either in very young patient or very old patient, but they come with medal. This patient has a most concentrated ener. I can some Vues in the cytoplasm as well. Okay, good point. Therefore, I say this patient is HIV positive. Is it going towards Burkitt? It is. You can see there are multiple vacum in the in these blast. Patient has a single large mass in the growing area and I have given you a clue as well that the patient is having HIV positive. If it is a dlbcl, he would have wi spread lopy all over in the body. B INF does not come usually with. It's very unusual. These cells are very, very basilic for some reason. This slide is lightening a lot under the scope. I don't know why, but they are very bopic and you can see some of the cells has vaces here. Patient has a single HIV positive. It is most likely Burkitt. I'm saying most likely Burkitt because because you need to confirm your diagnosis on flowetry and cogenetic. What is the expected flow in work? Positive for mature belel markers like, uh, surface imog Global in CD1, 1920. C will be negative. Oh, sorry, 20 C negative, CD9, 20, 22 positive. So CD1 is negative or positive? I think it's positive for for BS. The dlbcl, barket, and fular, they are CD1 positive. Rest they are negative. This will be CD5 negative, CD10 positive. What about bcl2 and bcl6? Bcl2 negative, B6 positive. This section contain a lot ofes, but I don't know why there is, um, too much light on this slide. Otherwise, you would I've seen a lot of regulation here. Remove the condenser. There's no condenser here on the microscope. There's no condenser here. All right, but I don't know why too much bright. Maybe now that's better. That's better. I don't know why this slide is too much lightning, but you can see this is full of ves here. Listen, too much bopic, a lot of speculation under the microscope. This very better, an excellent to see, but C here, the camera. These are all V. This line whole is vacu. This section is vacu. Um, I don't know how to make this more clearer, but you have seen now a lot of speculation. Yeah, that's that's perfect picture now. Straightforward. Yeah. All right. And what is the carot type or translocation in bucket Loma? 8:14 plus 822, 28 and 28. All right. This is a young man, um, with diagnosis of B lymphoma. Do we want to treat? Would you like to treat per or come on guys, clinical hematologist, would you like to treat bureta or not? Yes, yes. You have to start the treatment as soon as possible because this is one of the fast growing lymphoma, high grade lymphoma. The patient will die in two days. Do not start treatment delay. What you need to consider in buret while treating the patient? Are you worry about anything? Iope of tumor liis. Tumor liis syndrome because they have high tumor burden and as soon as you start any regimen for this patient, if it is AR or arodo m i, the patient will start responding to the chemotherapy and the risk of is high. So better to be careful about. Right. Is there anything you want to ask? Any question, sir? Can you please guide on the thing that, how do we go about the preparation? Like there is coagulation on one hand, then there is, uh, oncology on the other hand, then we have RBC pathology. So how should we proceed basically? Should we do everything simultaneously or something system we should pick up like that for the part two exam, you mean for part one? For part one, in there was a session last week, how to prepare part one. Did you attend that session or? Yeah, I attended that session, but I just wanted to ask he, we should study everything simultaneously or we should finish off coagulation completely, then we should go to the other this thing. Okay, so when I was studying for part one, I used to study weekly on weekly basis. One week coagulation, one week transfusion, one week oncology, one week gender. I would repeat it like that so that everything is in touch. Secondly, we are doing essays in our part one group. There are 300 people and I receive only five answers. That is a normal thing now, but if you can practice those essays, then we discuss these essays on session, except for those Saturdays and Sunday we are on call and cannot discuss. And we share MCQs in the part one group as well. That will be your MC practice as and okay. If you had listened to the part one session last week, it has more information. If you are in a part one group, hopefully you will be ready foring. Don't worry about. Okay, okay. Thank you. Nothing else, then we will close the session now. And if you guys can donate to maintain my site holding fee, I would thankful. I will share the link in. Have a nice Sunday. Take care. Bye-bye. Thank you very much. Thank you. Thank you. Appreciate it. You.