Transcription
Hello. Can you hear me?
>> Yes. Start soon. All right, let's start now and the screen is also visible to everyone, right?
>> Yes. Yes. Okay. So if there is anyone waiting in the gallery, please admit them. Right. So this is the first case. Um, this is a 56-year-old man referred by GP because of persistently high white cell count and he has eczema as well. This is power 10 for you guys. And now we will go to power 50 for this patient. Yeah, this is power 50. And let's see the sun. Right.
[clears throat] So I would like you guys to report this blood pen please. Anyone who want to make a start, you are the first on the on the list.
>> Sure, I I'll give it a try. So there is an increased population of a typical lymphoid cells. These are moderate in size with irregular nuclear outlined clump chromatin and some with prominent nucleoli. The >> cytoplasm is basopilic um with no granule scene. The red cells are mostly normocchromic, normocitic. Um, I can appreciate many with how jolly bodies. Platelets are are largely unremarkable. My impression for this case would be someone with a lympho proliferative disorder likely syndrome in view of his clinical history.
>> So what does the clinical history tell you?
>> So he has eczema. So I'm thinking something with skin infiltration.
>> Mhm.
>> And the irregular nuclear outlines are highly suggestive of a cutaneous T-C cell lymphoma. Yeah. So this is also one of the trick in exam as well that you can figure out from the scenario that what you are dealing with. They mention to you that this patient has lymphocytos luccoytosis and eczema as well. Okay. But it is not always the case. It may be a simple luccoytosis as well in the exam. But if they give you a hint it is very helpful. Yeah. So these nuclear features that this patient has is clums in a cerebr way. Uh your suggestive of a limp proliferative disorder likely scissoring.
>> So can you complete your report please?
>> So I would suggest to send peripheral blood for flowcytometry. um
>> for further staging work up um with a PET PT if they are lift adenopathy and to arrange for a bone marrow aspiration and refine for aspirate um uh and for aspirate and cytogenetic and refine biopsy.
>> Okay. All right. So you have commented on the um cell line your impression and your suggestion as well [clears throat] and um next question would be what is the expected flow in this patient? So it would be someone with a T- cell lineage. So, uh it'll be positive for TD3, TD CD4. Um I'm not too sure about CD8. I'm not too sure.
>> Yeah. So before we go ahead, so this is the typical cells of scissor. I think you cannot find a better cell than this one. I will make it a big for you. It has typical typical brain appearance. So you cannot find a better cell than this in in in your practice. This is a not unequal slide. This is a patient slide. Um [clears throat] but these are the typical features of scissor syndrome. Uh the blood features. Yes. So they what about the close automatic? What were you saying?
>> So I will do T- cell lineage flow. So CD3, CD4, CD5, CD7, CD8.
>> Mhm. They are CD4 positive or CD4 negative.
>> I think they're CD4 positive
>> and CD8.
>> I think it's CD8 negative.
>> They are CD8 negative, CD7 negative, 26 negative. and they will have T- cell phonology as well. How would you treat this case or how do you treat scissor syndrome in general? This this will be your third third case in the exam. So treatment of cesari syndrome first we have to stage the patient um and treatment in this case with blood involvement um likely the marrow is also involved. So he would probably need systemic treatment um which is chop based. He has a skin involvement eczema. So topical treatment in the form of steroids then systemic chemotherapy because blood is involved and bone marrow is involved then it will be top based chemotherapy. If they are refractory to that then we have ECP extraordal protopharesis. There is no typical BSH guidelines for scissor syndrome. Um the one that we study is all from Ash where where they include some advanced chemotherapy for scissors but we usually use job based therapy for systemic and corticosteroids topical therapy as corticosteroids and next line is photopheresis.
All right. So this is your scissoring case. These are the basic questions that they ask you in the exam. If you have appeared in the exam before, you should continue practicing um morphology because morphology will be different in your next exam. This is the next case. This is a one one day old baby whose blood has come through to us. His hemoglobin is 77. White cell count is normal. Plated count is 400. This is power 10 for you. In exam they can give you plyover blood film as well. They can give you they can give you new native blood film as well with increasing number of candidates. Obviously they will increase the um level of exam as well. They will make the exam more hard. They will bring new things for you. So be ready for such type of scenarios in the exam.
>> [clears throat]
>> This is power 50. Yes. So, Reika Reika is next on the uh list. Rea, what do you think? How would you report this blood film if you were sitting in exam? Okay, then Habib is next on the list. Uh, Shabbana Shabban sir.
>> Yeah. So
>> okay.
>> Yes. uh so it's uh seems RBC morphology is an isopylocytosis is with macro macroytosis polychromasia hemolytic picture and there is nrbcs I have seen and fragmented RBC's whole jolly body I have seen few target cells um otherwise platelet counts are normal and WBC is also looking normal in morphology ology. So, uh yes, NRBC I mentioned already and um so it's looking like hemolytic anemia. So, we need the history uh of the baby. Uh what history do you want? He is one day old. He was born yesterday. means um the patient has means my impression is most likely HDN maybe because it's newborn baby and hemolytic disease and patient has anemia severe anemia and there is himolytic picture so it's looking like hemolytic disease of newborn
>> so I will do the
>> yes so I will do ker further testing for the a full blood count already done. So I will do the that and Billy Rubin um uh and all hemolytic workup with group and screen. So I will go for the urgent maybe for the newist review for to start for the exchange transfer or topup transfer accordingly.
Okay. So you have commented on the red cell line because red cell line is affected. Uh you have noticed white cell as well but you didn't mentioned white cells in your report. What about the platelets?
>> I I mentioned white blood skulls white blood cells are normal in number and morphology and platelets are adequate. I reported already
>> there were few rectile lymphosytes. This is a brand home.
>> Yes,
>> there were few whole how jolly bodies.
>> Yeah, how jolly bodies also I mentioned.
>> Yeah, this is a reactive one as well.
>> A reactive. Okay.
>> So, you cannot say that on the blood film report that this is HDFN. You need a history for that. Okay.
>> You can say that this is
>> hemolytic anemia most likely consistent with hemolytic anemia. Please send the healic screen.
>> Okay.
>> So you have to mention all the names of the hemolytic screen component in this case.
>> Okay.
>> And that I will inform theologist Arjun. You are the hematologist. This film is abnormal. So in in practice you inform you call the neonatlogist that I have a blood film of one of your patient. These are the parameters. Okay. Please act urgently because you are adult hematologist. You you do not deal with have their own periodic hematologist.
[clears throat] M
>> so yes this is a picture of hemolytic anemia and the history of the baby is that the mom has antibodies and he has now developed a healic disease of newborn can you please mute yourselves please can you name few antibodies implicated in HDFl.
>> Uh yes. Uh the most common is um NTD uh and then um anti small C and um um K K is the most uh is causing the anemia. K is the more most notorious yes is the most clinical significant. All these three others also can cause but is the less significant
>> and the third question would be how is HDFN usually treated?
>> So HDFN um is exchange transfer they can go they can go for phototherapy um uh mostly or topup transfer according maybe. So these are the three options. In a sequence you you jump to extension transfusion then you come to phototherapy then you went to
>> to top up. So be in a sequence mild moderate and severe
>> mild one need observation moderate one needs topup transfusions and photo therapy and severe one yes they would need exchange transfusion. Okay.
>> Yes, it can be related. It depends on the history which will be known to the known to the neonatlogists. Uh we do not have much information whether it can be mismatch related himsis or Yes. So this is a third case again a 50-year-old patient referred to you by GP and this is over 10. He was referred to you because of the ilcoytosis. And this his his blood film. His white cell count is 132. His hemoglobin is 90 and plated count is 90 as well. His only complaint is fatigue. Sorry, what's the
>> 56? So this is power 10 and now power 50. You will not expect such film in exam because exam findings are not that obvious as it is in this in this patient. This is a patient blood film not from Nikos. Yes. Um, Denia is next on the list. Uh, Sabrina Habib. am. Yes.
>> Yes. Um can you report this blood please? H from RBC point of view it looks normic normocchromic mainly and thromocytoenia it looks decreased from WBC's point of view there are abnormal blastl like cells mostly looking mature lymphoid cell sometime with mature clumped nucleus but cytoplasmic blabbs. All of mostly showing blabbing of [clears throat] now on higher magnification. Some of them are indentation, nuclear indentation showing with cytoplasmic labs. Smear cells also can be seen. Mhm. What is your impression? Further testing needs iminohistochemistry flowcytometry to confirm the diagnosia lymphop proliferative to differentiate in between these two entities.
>> Right. So you have commented on the white cell count first because they are abnormal then you higher higher liquid county showing [clears throat] mostly abnormal cells then you have commented on red cells then you have commented on on platelets because we comment on the abnormal cell line first. Right cell uh white cell is affected which is the prominent one here. So you have commented on white cell first you mentioned there are bloods there are um lymphosytes with prominent nuclei smear cells and then your impression was lymphop proliferative disorder and then you mentioned that this patient would need peripheral blood sending for urgent flow cytometry in this patient and because he is anemic as wellic as well he would need a bone marrow biopsy as well to confirm this is your your report which is good. What is the expected flow in this patient? Which lineage will be positive in this patient? T- cells or B cell? What do you think?
>> Oh, B cell.
>> Why it would be B cell follicular? No, not for this is blabbing. Actually I couldn't recall the flow or typical morphology of which type of
>> Can I maybe sir can I add?
>> Mhm. Sir um what I remember is cytoplasmic blebing with lymphop proliferative disorder is most likely coming with tlnll but in the scenario uh I think scenario you mentioned any eons or anything I don't know I'm not sure
>> it it does not mean that if the if the patient has tlll he will have eusions as well but yes
>> okayes plural eusions they are part of the disease process eusionites they can come with proliferative disord you will have seen a lot of molecular patients with plusion a lot of marginal zonoma patient with refusion or recites um
>> so we will go for the T panels and then
>> yeah so T panel will be positive CD7 will be very positive here
>> yes CD7 CD2 3 5 can be positive and CD52 uh I will see for the treatment point of view.
>> Yeah. How will you treat this patient?
>> So again sir I will answer Dr. Mubashar is the priority because this is his case.
>> Okay.
>> He has passed the question to you guys. Okay. So um I will uh go for the means it's if symptomatic patient a patient is already been means is WBC very high hemoglobin and plated low. So it's cytoenia. So I will treat as we can treat as if 52 positive lmap and we can see the fitness of the patient uh and we uh we can go for the transplant in first remission. How to bring down the white cell count low?
>> Uh yes. So in that case we can go for the lioperasis in our joint. Yes.
>> Because there is no effect in this patient. He has no headache. He has no
>> uh blood vision.
>> Can we wait for that patient Dr. Rome because he is not symptomatic. So is is that is that good to wait for symptoms?
>> He has fatigue. He um he has ongoing tiredness.
>> Okay. So he counts.
>> Okay.
>> His count is very high. He's anemic. He's thromocyetenic as well. Um altismab may take few days to prepare. You may be waiting for MDT as well or Bulma biopsy result as well. You cannot start it today or tomorrow. It may take few days. But how would you bring down the counts?
>> Steroids. We can give that steroid
>> trial of steroids but in
>> yes
>> for some some reasons TPLL patient usually do not respond to steroids. Um we give them a trial only because we have many TPLL patients but we give them steroids but council does not
>> cytored reductive therapy. Cytoroductive therapy. Do you think it will affect the lympoid lineage? It affect the myoid lineage only. If the myoord lineage cells are high um then cyto reduction would work. It does not work on the lympoid. Um so yes a tried of steroids you can use here but the response rate to to steroid is not that good. Yes, the treatment is alismab maximum 18 cycles of altism up to the patient and That's not in frustration. This this one is a 60-year-old patient with progressive anemia and abdominal discomfort referred to you by GP. Because GP is the primary person here in UK health care system. The blood pins are referred by GP to the specialities. This is our 20. And this is Yes. So, what do you think is going on in this blood film? anyone anyone can shout out. It looks a smear of pansyenia progressive mostly RBC's are actually dimorphic pictures some RBC fragmentations macro sites um teardrop cells also there maybe some poly chromatic cells also are also there in noviceses it looks like that but at 20 I couldn't appreciate much may need to go on 100 resolution. uh there was p there is also thrombocytoenia and white cells are also low only I [snorts] can appreciate one white cell that was uh I couldn't appreciate it's normal or abnormal with lineage that was actually there was one nucleated red cell also seen is there any abdom ultrasound or any other no ultrasound. So this is a hyper segmented neutrfil some prominent granulations also. What is your impression? Maybe simple megaloblastic anemia the nutritional nutritional anemia or we need to work up for baro if needed rule of milo fibrosis.
>> Okay. Even uh this hyper segmentation can be due to displasia or MDS. We have to rule out also my plastic. Starting from nutritional better to go go with the workup for galloplastic anemia then further reticular side count will also we should present or look for is there anyis or anything cytoenia will lead to nutritional deficiency or while displac for a long time actually I'm out of this sessions and teaching say sessions that's why maybe I'm some not coherent or organized but I'm trying inshallah this is Can we go on handed for this sale? Large granular lymphoid cell maybe or abnormal myioite can be even dysplasia as shown. Mhm.
>> [clears throat and cough]
>> This is same cell.
>> Yeah.
>> Or same.
>> This is same cell.
>> Yeah. I went back to 50.
>> Yes. Yes. This has cell fragmentation large thrombocide platelet.
>> Yeah. If you are talking about nutritional what do you think about nutritional B12 iron? B12. No, no. B12 flet most probably.
>> But but there are lipid.
>> Uh there are liptoides also cells also.
>> They are tear drops as well.
>> Yes.
>> And this patient has abdominal discomfort as well absorption. Do not assume what is
>> do not assume in in reporting or for exam purpose that the patient may have abdominal patient may have malabsorption
>> sir can I get
>> mhm
>> sir I agree with Dr. Mubasher for all reporting. He mentioned everything in WBC, RBC and plate morphology. He well described pensytopia with all findings. My impression is according to the history 60 year and he has abdominal discomfort and progressive anemia and now tear drop in the blood film and NRBC liortolastic picture like my one I have seen and so it's most likely going for MPN is myop fibrosis for me so I will go for further testing like bone marrow aspirate refine biopsy with reticulin stain and of course cytogenetic molecular NGS panel.
>> Okay.
>> He has abdominal discomfort. They cannot tell you in the exam that this patient has spleen. Then it will be very easy for everyone but this patient has pensenia and spenagali. So it is either CML or it is either a cell leukemia or myop fibrosis. Yes,
>> then it will be very easy for even the FY1 can pick that up as well or the clinical scientists can pick that up as well. So they will give you a V. This patient has abdominal discomfort and he has anemia. This is the blood film. You have noticed that this patient has pensy with large platelets and abnormal red cell morphology. one nucleated red cell up till now we have seen there are a few um dysplastic neutrfil as well but this film is very pensic so it's difficult to find them in this film
>> so likely it's going toward MPN
>> MPN
>> but to confirm to confirm that this is MPN because you cannot say that this is my fibrosis on a blood film blood myophibrosis has its own criteria of diagnosis which include bone marrow as well and other investigation major and minor criteria.
>> So you would suggest all those tests and I will do bone marrow biopsy in the patient. I will check the LDH in the patient. I will exclude BCABL and then as per WH 2022 if the major and minor criteria are complete then this patient will be labeled as milofrosis.
>> Yes.
>> So what is the significance of iptoides? There are many iptoides along with the teardrops. So whenever the spleen is big you can have different morphology of the red cells when they pass through the spenic pul okay
>> they become teardrop they become electroy sorry
>> this patient is transfused that's why you are seeing um morphology or diorphic morphology in the Right.
>> Can I ask is is there macro sites because I can't really seem to appreciate because I think one of the differentials given was megaloblastic. I I which are the macro sites.
>> This is the macro site. This is the bigger cells.
>> So that's part of myop fibrosis. Dr. Amir
>> and in this patient later on we find out that he has poly deficiency as well. That's why he had macro macroytosis. That's why there was one cell that the candidate picked up was hyper segmented. That's why there is element of microytosis here as well.
>> Thank you. So how would you treat this case? If this is a myop fibrosis for example we had he had a bone mar biopsy and then it shows that there is myop fibrosis he's anemic he's theocytoenic how would you treat
>> can I go sir
>> yes
>> sir I am because it seems a high risk patient is has pensytopia with symptoms. So uh I will first check the transplant eligible or not eligible. If it is um eligible I will go for the jack to inhibitor. Once patient will get the maximum response then we can go for the alograph and if the patient is not fit then we can continue the jack to inhibitors. um and if it is not responding at least I think 3 months we can continue. If it is no response then we can go for the second line medication or clinical trials and supportive management of course according to the pensytopia blood transfusion and folic acid like you mentioned is as holy deficiency and um blood transf of the patient if hemoglobin low plate low transfic antibiotics. Mhm. You do the scoring first in this session. Which score do you use for my
>> So dips. Dips plus scoring.
>> Yeah. Dips plus scoring. I will go for it.
>> Yes.
>> Uh yeah. And then I will of course risk assessment and then fitness.
>> Yes.
>> Yes.
>> Find out whether he is low risk, intermediate risk or high risk.
>> Yes. We check the EPO level in this patient, hematenics in this patient. Our patient was folic acid deficient. So we give him folic acid. We offer him EPO as well because he was in the low risk. But if he is a intermediate risk or higher risk, you can offer him treatment in the case in form of Jack inhibitor first and then transplant or directly in the form of transplant if he is high risk and donor is available. Which jack inhibitor would you use here?
>> Roxalatin. Can you use any other one as a first line?
>> Uh, molinib.
>> Okay. Is there any other one?
>> Uh, there is a trial of prito and federinib is not using now because of the veric and capopathy.
>> It's pecritinib. Fbritib is something else which is used in trial form in CLL in in in world and stone. It's a different medication petro approved.
>> It is not approved here in UK but it is entry is a nice approved I think 2024. Yes, but now it is less used because of the
>> lucine syphilopathy and now because the um that one is available now is available that's why
>> the new one is
>> new one is what
>> yeah Yes, spec is not BSH sorry NIC approved yet. It's FDA approved but not yet NIC approved. Hopefully by next year it will be there.
>> Sorry. So in the UK cuz I I don't work here. So you can choose either rock solitinip or malotinip as first line or do you need to fill rucks before moving on to malotinip?
>> No more malotinip can be used as a first line.
>> Okay thank you. Any jacket of inhibitor can be used as a person. There's another 60 year old patient referred by GPTU because of luccoytosis. He has a background of seriatic arthritis. This is power 10. Power 15. All right. So, what do you expect in this patient?
>> Yes. Can I go for
>> Hello.
>> Yes. Go on. Okay. So these are mostly the normocetic normocchromic fuse show the hypocchromia in the RBC. uh and in terms of the WBC uh there are the few um lymphocytes that are um mature lymphos with the medium to large size cell with the high density ratio and abundant cytoplasm with the granules inside.
>> Mhm. So and the platelet morphology is and the count is fine. Um your toes are present. My impression is the lymphop proliferative disorder. Um most likely large familial lymphocy lymphocytosis is LGL. use myself.
>> What is the hint in this scenario?
>> Because there is the autoimmune disease along with that rheumatoid arthritis the patient is suffering from.
>> Mhm. any autoimmune disease in the patient with they would have given you full blood count it would show neutropenia
>> another is the neutropenia as well
>> these cells are quite a lot in these in this film but in the exam film there will be only few cells you have to look for that okay so yes likely lymphop lymph related disorder likely LTL but you need to do further test to confirm that what is the expected flow in this patient
>> so that will be CD8 I think positive and TCR alpha beta rearrangement will be there and I think uh in few cases there will be the delta gamma tar rearrangement as well but I'm not sure alpha beta
>> oh it's not the delta gamma okay
>> okay so and the treatment wise is
>> patient is um you can say that the patient is showing the neutropenia so it's been if there is been there's any complication with in terms of the neutropenia then we will treat the patient otherwise I think uh just to increase the count we can give the patient GCSF a neutropenia Yeah.
>> Yes. If he has no other symptoms um related to LGL or only very low neutrfil count, you can first try GCSF in the patient. But if he's not responding to GCSF and he has other symptoms as well, then what are your choices of treatment for? then I'll go for immunosuppressive therapy for that because it's in autoimmunity. So
>> I don't remember but it's imunosuppressive and cycllosporin
>> okay lo methodate
>> okay
>> or cycloporine in the in the with or without um GCF These are common short cases that usually appear in every exam in a different case. If you have any question please ask
>> Dr. Amir when they ask us for flows bytmetry results do we just give the positive ones or must we also write down what is expected to be negative
>> the important negative ones as well.
>> Okay. Thank you.
>> Right. Whether this will be CD4 positive or negative, CD8 positive or negative because you have different T- cell leukemas which are some are CD4 positive, CD8 negative, some CD8 positive, CD4 negative, some are both negative. So they would be interested to know these the important ones only not everything. Right. Uh, thank you everyone. See you next month.