Transcription
Can you see the screen?
>> Yes, I can.
Right. Okay, we will start our session. This is the first case and uh this is 22 year old female presented to emergency department because of high grade fever and body X. The the full blood count is Hemoglobin is 140. White cell count is 18 sorry 22 and plate count is 336. This is the blood fin at 10 power. Now add power 50. kindly admit the people and open session.
All right. You have seen few cells. If you're asked to report this blood film for the purpose of part two exam, how would you report that? Anyone? I need anyone volunteer to report this blood thing?
Uh do we share it or uh do we just um uh speak out the answer um yet?
>> Speak out here.
>> Um so I think mild rulosine red cells appear mostly uh uniform in size normal cellular compared to mature lymphocytes nucleus. Platelets appear um adequate with mild with an isocytosis, pleomorphic lymphosy seen. Um some um reactive forms and some larger atypical forms with um basophilic cytoplasm. Neutrfils appear left shifted. Um my comment would be film appears reactive uh needs ruling out um infections such as viral causes. Um so to rule out uh CMV, EBV, hepatitis uh viral screen um and clinical assessment for uh hepatitis uh repeat film in about four to six uh weeks time to assess for resolution.
Why it is not a malignant blood fin? What makes you think that this is a reactive blood fil?
>> Um, I mean to me lymphosytes are pleomorphic. Um, they're not exactly monomorphic. Uh, the nucleus still looks a bit condensed. It doesn't look very open chromatin. um and uh in the age group if I would have to take a guess um particularly if it's a very acute presentation with no other B symptoms I would first like to also rule out uh while considering clinical assessment uh infections um and also the neutrfils look left shifted which goes more towards a reactive ideology.
Yes, the cells are pleomorphic and the age group, the rule of formation with normal hemoglobin uh they all goes in favor of a uh reactive blood film. This this blood film is very common in exam. It appears every second exam and um people make it all, AMLS, buckets, different forms from it. But uh this is a reactive blood film or infectious monucleiosis blood film you can say because we have to do the uh monosport test in this case as well. cerology. You have done a viology which is good. You have to do the hepatitis BIV cerology as well and the monosport test.
All right. So even if you um make the diagnosis wrong but you have commented well on your answer, you you still get marks. I will show you one picture. This is from last exam. Okay. You can see um this candidate here score uh 9.5 and here the diagnosis was wrong. Which means that if you make the diagnosis wrong but your report is correct and you answer the remaining question as well, you can still score marks. See this candidate has scored 9.5 even the diagnosis was wrong. Right? So if you make the report correct like first the comments on a cell line and then impression and your suggestion which you did in your report your report as well and then the next question would be what further investigation you would be doing. So if if you have answer that this is likely a reactive blood thin I would do viology monosport test as BC and look for and blood culture to look for the cause of infection you this is correct answer but if you have mentioned I will do bone biopsy flowmetry this is wrong and the treatment or management of this case is conservative right? So I'm sure you would have done your Um uh you you would have made your line box. Keep the reactive blood film must cuz it appear in the exam every now and then.
This is a 9-year-old boy um who presented to emergency department because of feeling unwell and when they did the full blood count on this patient the white cell count is 600. You can see that as well. The patient hemoglobin is 90 and the plated curve is 90 as well. And this is the power 10 which is full of cells. You can see that And this is power 50. So any thoughts about this blood film?
>> Can I try?
>> Yes, please report the blood film.
>> Okay, um uh the blood frame shows u marked luccoytosis uh with uh small to mediumsized uh blast which has uh high NC ratio um uh open chromatins prominent uh nucleoli um uh blue blue cytoplasm um mostly a granular occasionally granular um And then there are multiple smear cells present. Uh there is mar thrombocytoenia neutropenia and uh uh red cell count is also decreased. So um uh uh it is likely an acute leukemia most likely acute uh lymphoplastic leukemia. um uh so correlate with uh flow bone marrow aspiration and refine biopsy uh cytogenetics and molecular studies and uh um um I suggest uh if it's um uh patient might read urgent um um start of the treatment um with steroids after flow confirmation
What is the likely diagnosis here? Do you have any guess on that?
>> Um, it's likely NLL. Um, could be TL or BL might be TLLL because cytoplasms some mirror forms um hand mirror forms I can see here now.
>> Yeah.
>> Yes. So these are typical hand mirror.
>> Yeah. which can give you a clue that this can be most likely um TL but you have to do closemetry on this blood sample you have to do bulb biopsy to confirm your diagnosis. So if they give you a flow that they say CD2 positive, solo plasmic CD3 is positive, CD10 is positive, TDT is positive, CD19 negative, 20 negative, you will be thinking that this is a um then um CD 19 is uh it TLS. Yeah. Yeah, 1920 is negative. So B markers are absent.
>> This is most likely TL.
>> Yeah.
>> If you are very sharp and know about the subcategories of TLLL, then
>> yeah, it's a pro like a pro TL. Yeah.
>> Um the next question on this on this slide in the exam would be um what are the poor prognostic factors in this patient? So the poor prognostics is the high count as is more than u 100 um uh then um it is um the cyto um cytogenetics and molecu molecular associations um if it's like apart um and also like if it's um uh early precussure tll L or uh and also like the MRD u response uh response to the st response of the steroids uh and if it's a male so um like if there is a CNS involvement testicular involvements
>> any mutations uh in the TL cannot recall I think um uh it's a um it's a tall like [snorts] or receptor or hawka I think ha I think always remember complex carotype TP53 hypodlody they are the u risk cytogenetic if you do not remember any transllocation any mutation remember 53 3
>> which is which which is devil everywhere complex carotype and hyper diplo these three these are the important thing if you forget in exam in viva in paper you can include these three things in the poor prognostic factors
>> how do you treat it
>> uh so urgent admissions um and then um after confirmation of the diagnosis start um steroids um uh uh pre-phase steroids and then MDT discussion um and this share decision making with the parents TLS profile access um and then um we have to go for the pediatric protocols UKL um pediatric protocols along with narrin There is all together protocol.
>> All together protocol.
>> You know about that, right?
>> Yes, you forgot about that. Yeah,
>> there is all together protocol and you would be offering this patient all. You do not need to know the details of all together protocol. Um but TLLL itself is a intermediate risk and poor risk category in the all all together protocol um subcategories. Okay. Um they would not ask you about the details of it. It is a periodic protocol and uh they deal with that. Okay. But you should know that you have altogether protocol which we offer to the patients in UK up to the age of 25. All right. Uh from age 2 to 25. In other European countries, it's up to the age of 30. They may ask you what what would you do in relapse setting? Uh [sighs] not very sure. I think relapse settings um if the we have to um think about a narabin or and then the stem cell transplant.
>> Mhm.
>> Yeah. Or carti therapy. Not sure about the T cell. I think it's more for the B bli.
>> Yes. In UK we use narin.
>> Yeah. And uh we also give them um pl IDA as well to to empty their marrow and go for uh stem cell transplant. Mostly it is narabine because the prognosis is very poor for the TLL especially the relapse TL. um all the patient may not get allergenic stem cell transplant but they may not ask you this much detail because it is a periodic thing and as adult we do not deal deal with it but remember the altogether protocol and it's inclusion and exclusion criteria because they may ask you in the while your patient may be 20 year old patient you still can offer them all together. We use all together in our adults patient as well.
>> All right.
>> Thank you.
One of the candidate the other day asked me do we do cyto reduction in in those cases where the lymphocy count is high. Anyone do we do cyto reduction in lymphocytosis?
>> I don't think so.
>> I thought my Yeah, my understanding was that we usually very steroid responsive. So you need to actually monitor them for TLS once you start steroids.
>> Yes. For example, you have a CLL patient or you have follicular lymphoma patient or you have a TTL patient and the count is 400. Would you do cyto reduction in them?
>> No, I think the cyto like we use the steroids if we think that they're having problems. The starter reduction is done in in uh if if the plated count is high, if the red cell is high, if the myoid side of the white cell count is high, if the lymphosy count is high, we don't do cyto reduction. We give them steroids only. Okay? If if required, right? CLL patients may have 300 white cell, but they may be asymptomatic, but don't do anything with them. TL patients if their white cell count is 700 and they are asymptomatic we don't uh do cyto reduction for them we give them their own treatment if indicated um which is methtores okay so cyto reduction is only for high red cells high clicked count and myoid type of white cells or lymphoid type of white cell if they are high we don't do we don't give them hydroxyarbomide we don't do any sle reduction in them. Yes. If if the nymphocide count is high and they and and they are causing complications in the form of pulmonary edema or or incraanial hemorrhage or clot then we apheresize them to reduce their um number. Okay. But hydroxychamomide or other form of cyto reduction is not done in nymphoid type of white cell count if they are high.
This patient, this is a 50-year-old patient with a background diagnosis of DLBCL and he has completed the first cycle of um polar archer. Okay, he has bloods done at the end of the cycle. And the lab noticed that the full blood count is high. Blood count is showing hemoglobin 120, white cell count of 30 in which neutrfil count is 28. Clicted counts is normal. This is power 10 and this is power 15. Now SL. Go back. You have seen many GCSF induced luccoytosis. You need to report the blood pin.
>> Okay, Uh the red blood cells um shoot an iso policy. uh there are microitic hyperchromatic picture along with the few polychromatic cells target cells and microspherosytes and white blood cells showing gluccocytosis with the predominance of the neutrfils and the brand forms. There's no any atypical uh lymphoid cell or blaster cells and the plates are adequate and spare no clumsy. The findings is as the patient is post chemotherapy. So the findings are suggestive of uh GCSF induced.
Um what suggest that this is GCSF induced neutropenia not infection or not chronic neophilic leukemia?
>> there is no any uh toxic granulation. It can be differential. The infection can be differential but there should be some kind of a history of fever or any clinical history that is not given this and there should be some kind of a toxic granulation or evacuation in the neutrils. there are many granules here
>> and the nutrition.
>> Yeah. Okay. So this patient they have given you a history of DLBCL. They mention that the patient has finished first cycle and once the patient has first cycle of chemotherapy art they use GCSI for 5 days. Okay. So here um the
>> there is a myoid series of cells here there are band forms there are naturophils there were few milocytesAS WELL um and then the red cells you can see they patient is a bit anemic but we have seen many target cells many stomach metites were there as well even and the plated is okay. So in absence of any infection history this is most likely GCSF related. Okay. The next question can be what are the differential diagnosis here. Okay. In the differential we can
>> sorry we cannot hear you.
>> Can you say it again? We cannot hear you. Are you there?
>> Okay, we have lost.
>> Uh yes, we can say that we can put this differential like in differential we can put the infection and chronic leukemia down the list.
>> Mhm. Yeah. So this case has appeared
>> multiple times uh previously uh in the exam. Uh even this was my case as well in my exam in uh 2024. Um a patient they have not given us the history of DLDCL. They have mentioned the patient is complaining of pain and that pain was because of the GCSF injections. white cell counts were high and um plates were normal. GC hemoglobin was normal and there were a lot of neutrfils in the in the blood fin and um they asked us to report the blood fin what are the differential diagnosis what is the management etc. Okay. So, GCSF has appeared before. It will appear again. So, please make your own blood films for the GCSF induced neutral luccoytosis. Chronic neutralic leukemia is very rare but it is present. Uh recently we have one patient of CNL. Uh it it blood film can appear in his hand but in that all the neutrals are mature uh in CNL. Here we have seen bend forms. We have seen milioides. We have seen few mature neutrfil as well.
Dr. Ramir, is there underlying any red cell disorder in this patient because there's a kind of lots of target cells and some sperosytes.
>> The patient has a liver disease. uh that's why there were a lot of target cells in this patient.
>> Okay. Mhm.
>> So you have to mention those um abnormalities in the in the blood pin like you have noticed that there are target cells. You have noticed there are a few stem sites you have to mention that in the in the report. The diagnosis may be something else but but these are the you have to mention it in your report.
This is another 50 year old patient. Dr. Can I check something with you?
>> Yes.
>> Yes.
>> And the uh red cell effects be as a result of chemo effects.
>> The red cell effect. Sorry, what?
>> The red cell feature. Can it be as a result of the recent chemo that the patient had?
>> Yes. The chemotherapy can affect the liver and if the liver abnormality is aggravated patient can usually develop uh target cells. Once the liver is okay target cell disappear from the liver. One of the reason of target cells are the liver dysfunction.
This is a 50-year-old patient um presented to uh GP for a routine blood checkup. And the GP noticed that the white cell count is high. Lids are 120. Hemoglobin is 130 and the white cell count is high. And you can see they are more than 100. Okay. And this is the power 50. All right. So, any thought about this blood film? anyone. Any thoughts about this blood? If no one wants to try it, I can give it a try if that's okay.
>> Yes.
>> Um, so there is a prominent lymphocytosis which appears to be dimorphic populations. Some are small mature cells. Some are medium-sized with somewhat open chromatin um and prominent nuclei. Um these are of various um there is a variability of size and shapes. Uh here um we'll stop then red cells appear reduced. There is no obvious polychromatia or spherosites that I could identify. Um platelets appear reduced with nizocytosis. Uh occasional monocyine and neutrfils appear reduced. Uh film likely in keeping with a new uh lymphop proliferative disorder. Uh I would advise GP to contact the patient um and uh assess them urgently for B symptoms and uh refer a sample for flowcytometry.
Okay. So, what do you think which name it is?
>> I It's so polomorphic. It seems like mantle cell to me. It doesn't look like CLL. Um and and that's why I think flow would probably be very useful here. Plus there is no not much of uh uh polychromasia going on in the background. Uh from a if this was a CLL you would assume that there'll be a bit of himolysis going on. So I think I would probably go with mantle cell.
>> Okay. Any other point?
>> Uhorphic mental cell lymphoma patient they are usually very ill but this patient is asymptomatic.
>> Asytomatic. I mean other thing would be if it would be the splenic leukemia lymphoma with prominent nuclei. So the ent the new WH entity which might have whether this is marginal zone or on on those lines marginal zone then if he's quite well
>> yes this is the splenic leukemia with prominent nuclei. It's a very rare case but I managed to find one blood film in 2025. Um now it is a combination of her leukemia variant and the BPLL. These two com these two entities are now called as SLLPN who is clinically formal leukemia with permanent nuclei.
>> Okay. Yeah,
>> previously it was hairy cell leukemia variant but this one doesn't look like hairy cell leukemia variant um because the cells has no projections. They have prominent nucleoli but um no hairy projections.
>> Yeah. Yeah. So this was a blood film. Very interesting one just to show you. We have no cases of SLLPN and only this one in the last two years and because the patient is asymptomatic we are not treating it.
So uh I can ask you so in exam would they put obviously they will probably go by um what's what would you consider next in terms of so if the patient is asytomatic and let's say if we are not sure of the diagnosis can we just leave it as uh like you know GP2 assess for B symptoms and refer it for two week weight um uh cancer pathway or
>> yes if the patient is asymptomatic then you will advise the GP that Please refer the patient to hematology service or two two week weight pathway. If the patient is symptomatic, please refer the patient urgently to us. Okay. But if the blood pay command says that patient is unwell, then usually we call the patient and ask how how is the patient? If he is unwell, then we bring him to acute assessment unit. But they if they have mentioned to you in a scenario and the patient has gone for annual blood checkup which means patient is well patient has gone only for blood blood review um as a routine then you will ask the GP if the patient is asymptomatic please refer him to hematology unit or twoe weight pathway if patient is symptomatic inform us urgently or refer the patient urgently to hematology. Thank you Amir for sharing a rare diagnosis.
Can I ask so let's say if I'm now that you have mentioned it so obviously with mantle they'll be sicker so they wouldn't be presenting with this count and GP they might be more unwell. So that would probably be one clue is it to think more of a more sort of a uh pro proliferative rather than sort of an more indolent disease.
>> Yes. If they are symptomatic it means uh the diagnosis may be something else. So for that purpose you need to do flowcytometry. Okay. The patient need to come to the hospital being admitted need flowcytometry to find out which lineage you are dealing with and what is the most likely diagnosis on flow. Then patient would need um bone marrow biopsies or lymph node biopsies um and the rest of the investigation for lymphoma and initiation of treatment. All right. So in this case if you don't know the diagnosis but if you have mentioned the this is a lop proliferative disease which needs flow to find out the lineage and most likely diagnosis your report is okay as I have shown you the the candidate uh result okay the diagnosis was wrong but still he managed to get 9.5 marks out of 10 and on on blood pin we do not expect you to write the correct answer because we don't make diagnosis on blood films. We can only say that most probable or most likely.
>> Thank you. Even for TTP, even for APML, we say most probable diagnosis is APML and TTP and still we send PML aurora stain or admts 13 profile to confirm that. Okay. And if required, these these cases are usually CD20 positive. Retoximma monotherapy is usually the equipment or if they have very big spleen and patient is symptomatic with the with the spleen then sometime radiations are offered or splenic radiations or splectomy after MD decisions but the start of the therapy in the literature because we have no guideline for SNLP in BSH is usually the rettoximal monotherapy.
Now this young man a 40-year-old went to the GP because of the abdominal discomfort and GP did blood test which shows white cell count of 400. And you can see the power 10 blood film which is all um full of white cells. Now this is power 50. [clears throat] Mhm. Yes. How would you report this blood film?
>> Can I try?
>> Yes. Go ahead.
So there is marked uh luccoytosis uh with marked increase in the neutrfils and left shift uh uh all stages of granocytes are seen including myioite metamocite promyocytes band forms neutrfils appears um morphology appears um sometimes hyper segmented um uh there is also a peak in the milocy series. Uh there are some circulating blast scenes which are large high and cy ratio um prominent nuclei. Um uh then uh there is einophils and basopils are seen with some einophilic precursors. platelet count appears to be normal I would say or mildly mild reduced and uh there is mild uh normatic normocchromic anemia nucleated red cells have seen um so uh these flames is suggestive of u myoprol chronic myopolifactive disorders likely a CML blast blastic phase after or um but needs to be differentiated and um I'm sorry it should be chronic phase I'm not sure blastic phase chronic phase so needs to be correlated with um um bonear respirations s and refines and cytogenetics, BCR, ABL, uh fish for BCL and RTPCR and uh cytogenetic studies. Yeah.
The patient is with GP.
>> Yeah. [clears throat] So, uh uh GP needs to refer I need to call the patient urgently. So, uh we need to contact the patient urgently about u uh um urgent assessment. Okay.
While in route to hospital, this patient developed headache and the CT scan shows incraanial bleed. How would you what would be your immediate intervention in this case?
um eating and international. [sighs and gasps] So uh so patients needs some hyd um hydroxyarbomide immediately to start with and might require um lucoperasis as well and um um will need to assess for coagulation profiles um u as well refer discussions with the neurosurgery ICU admissions stabilization of the patient not might require lucaresis the patient requires
>> will require yeah
>> because the white cell count is very high 400
>> and he has developed complication.
>> Yeah.
>> Which is most likely because the blasts are eating the one multimer. So you have to do liparesis for this patient to reduce the white cell count. One session is enough. It will come down to double figure and then you have to give this patient hydroxybomide to continue. And yes, neurosurgical opinion, ICU admission, that has to be done.
Then um when would you start the CML directed therapy in this patient?
Um so I need to confirm the f first with the the fish and then um we need to send for um MD discussion share decision making um I think we can start the TKI um like once the diagnosis confirmed.
>> Yes. Once the BCBL is um results are is available with you
>> you can start TKI if the because you would have done liferesis s reduction the white cell count would have come down to
>> 30 40s usually when white cell count is around 30 then we give this patient TKI
>> which TKI would be your preference here
>> um he's a young patient. So although it's a like a shared decision making with the patient um but I will possibly go for um dasatinip like second generation fastline TKI because it's more potent and because this patient present with kind of very high count and aggressive disease rather than anyone disagree with desertinip So I don't think so the white cells are the reasons for desertive I haven't heard the full question but at least the reason sorry for this
>> he say the the patient is young
>> okay uh 14 years old I think in that case yes But but um somebody in the audience says the sultinib is not the right choice in this particular patient. What is the main side effect of the satin? I think sex dysfunction
>> plate dysfunction and plural fusion I mean perunary side effect
>> yeah desaturation because of pericardial diffusion pulmonary eusion pulmonary hypertension etc.
>> Yeah. uh but because of the platelet dysfunction um most of us would not use the certain in this particular case.
>> Okay.
>> He can use normal imagatib.
>> Okay.
>> Okay. But we had a case like this and we discussed in the MDT whether to give the certain or not because if the patient has high ELTS score or major root abnormalities related mutations or in a young patient usually we we prefer either give desertib or notinib and MDD decided not to go for datinib because the patient has antrain bleed. and the certain causes plated dysfunction. So the bleeding risk will still be there. Then we decided to go normally with eartin as first pass line. This was an MD decision. Things may change. If you can give your reason during why you can use the certain if you have a reason for that in the W because they may question you in the WA the certain causes plate dysfunction are you still happy to use in the certain in this patient who has intrainial bleed few days ago. So if you have a reason for that then you can you can say yes. But if you have any evidence um where you have not used a certain in young patient you can quote your MDT you can quote your uh patient without identity um as an example. Okay. Right. Yes.
So these were five cases and it's 6:30. If you have any question please shout out.
>> Uh Dr. from in that this case it's a we can see there's a few blasts but it hurts to say without like differentials if will it be like big problems if I say is like is the chronic phase versus blast phase is wrong if you are able to count the blast percentage in the blood thin and they are more than 20% then yes it's blastic phase or you will be treating it as AML in combination with TKI but if it is less than 20% then as per WH 2022 it is still a chronic phase because we have no accelerating phase now so less than 20% is um chronic phase more than 20% it is a blastic phase you will be treating it as AML any question?
Okay, then see you on Sunday.
>> Sunday.
>> Thank you so much.
>> Welcome.