Transcription
e e e e e e hello everyone can you hear me? Can you hear me now? Yeah, morning. I'm here. Morning, everyone. We woke up and still going, right? So this is the first case. This is a 56-year-old who was brought to the emergency department because he was feeling unwell, body aches, fever, and no energy. The blood test shows that he has hemoglobin of 98, platelet count of six, and the red cell count of 13. The blood grows first to the hematologist or clinical scientist. Scientist or hematologist, understand this is just an artifact. Anyone from you want to come in on the question first? This is power 10, and then I will go to power 15. Okay. What? Okay. Okay. Um, there are spicules about. Yes, there are a few spicules. We will go to high. See. Mine, I need another. This one is power 50. Must be above. I say this. Sorry, what? Kill site? Kill site? Yeah, it's like helmet cells as well. White cell? Yeah, just side are cells and some cells. So polycythemia. Polycythemia. Maybe some. The patient is anemic. His hemoglobin is 98, but platelet may be dropped from more than 130. It was more than 130, then dropped. Maybe the result that we have got, it shows count of 13. Yeah, it's just the cells there. I think fragments. What's the coagulation like? Is the other film done? Yes, the coagulation profile was 1.9. 1.9, that's okay. Yeah, the PT and PTT were okay. Okay. Cause I was thinking it could be something like DIC. Could be autoimmune. Does not have this much of fragments. One thing I think the spicules, this one, this one has no, no white cells. One, two, three, four, five, six. That's why I said the this. Yeah, yeah. I TTP on the fragments like there as well. Yes, of them. B as well. PD, maybe G6 should have blood film. Yeah, yeah, that's right. And because of this much fragments, obviously the clinical scientist or lab scientist will ring you up. I have a patient with anemia, thrombocytopenia, and many fragments in the blood. Yeah, it's got to be in MAHA, some form. Then TTP, DIC. The PT is okay. There are fragments there. So it's healing. So now can't see any RBCs though. No, no. Put cells. So, so the clinical scientist thinks that this is MAHA, and according to our trust policies, such things are urgently related to the hematology consultant. Now I need a hematology person, hematology person to this blood. For the purpose of the exam, you are sitting in for an exam, and they have given you this and asked you to report it. Do you have? We don't have any clinical hematologist today. Russia is on the top of the list. How do you report this? Okay. Okay. Take that. But I'm from the laboratory side. Can I? Yes, appearing in regarding white, white. The cells there. There are unremarkable morphology on the abnormality. First, you have seen the red pathology here. Commence. Start with. Oh, okay. There is, um, anisocytosis, including, um, fragmented cells, spherocytes, polycythemia. Um, regarding platelets, um, mild thrombocytopenia. M. So I, um, I advise for hemolytic workup, coagulation profile, if it is not already here. What is your, what is your impression? Yeah, uh, my impression is the, uh, MAHA. First, first of all, MAHA count done. Count is high, right? I would go with it. But Dr. Said that platelet count is 130, right? 130. Okay. But there are nothing in to me. Maybe dropped from more than 13, maybe. But the first differential is MAHA. For cyclical coloration, maybe the patient has valve replacement or hypertensive. So I have to go for hemolytic workup and coagulation profile, fibrinogen level. Okay. So these things are usually done when a patient comes to ED here. When the bloods are sent, it includes full blood count, function test, liver function test, coagulation profile for the patient as well. This patient is normal. Sorry, they are AI. You should have asked me. This patient has anemia, a lot of fragments in the blood, few spherocytes are there. Decreased platelet count is reduced. No, um, no platelet flag up there. Most likely this is MAHA, and it can be a TTP as well, which is a hematological emergency. Yes. So this patient's investigation needs to be discussed with the TTP consultant or TTP center. 13 tests as well to be done. Yeah. So we need some history of this patient and other investigations like liver function test and LDH, and then we have to discuss with the TTP center. If the TTP consultant agreed that this is an immune TTP, and this patient needs to be blue-lighted to the TTP center, we cannot treat it in all the hospitals. If this is immune TTP and there is no secondary cause for that, then you have to blue-light this patient. If there is a secondary cause for that, then plasma exchange is not necessary. You will treat the patient in your hospital. Sometimes the patient is on cytotoxic mitigation, Tiger, Tiger, Gill. Sometimes they are on chemotherapy or post-transplant medication stuff. Sometimes they have any solid organ cancer, thentic cancer is the commonest one, and they come with this picture. MAHA picture. We do not do plasma exchange for them. Plasma exchange is done for thrombotic microangiopathy. If there is no secondary cause of TTP, and we, yeah, in hemolytic anemia, there will be polycythemia. There will be spherocytes, but there are no this much fragments. It can be only one or two fragments in the autohemolytic. Most of the cells in auto are poikilocytosis or spherocytes. This thrombotic microangiopathy has a lot of spherocytes. It cannot be ignored. It should be elaborated by the clinical scientist urgently by phone to the unall hematologist and call hematologist to discuss urgently with the TTP center to agree on the diagnosis of immune TTP. E. So in this blood film, I would like the candidate to list the abnormalities that you have seen in this blood. This is power 10. They're all there. The different myeloid series. I think now I will go to power 15 to list the, um, normal findings and then your differential diagnosis. From anemia, what else? What about these mast cells? There are no mast cells. So I asked you to list the abnormalities in this blood film. The mast cells. Okay. Parasite four times. These blood cells don't have M, otherwise they would have told you that I am NRBC, I am myelocyte, I am dysplastic neutrophil. Blasted picture. What is in the exam? In the exam, you will be asked to list the abnormalities in the blood film. Well, you got to start from red cell series. What do you see in the red cell? You mentioned there are. When they, when they ask you to list the abnormalities, then you have to write the abnormalities, not an isocytosis. You have seen stomatocytes. Write stomatocyte. Number one. Number two, there is spherocyte. Parasite is microcytic. List a microcyte. If there is any fragment, if there is, and there is NRBC here as well. List it as NRBC under the heading of red cell. Once you are done with the, um, the red cell series, then go to the white cell. And white cells, we are seeing here. Myeloid is there. This plastic neutrophil is there. Yeah, you will see more. Wait, hypochromia. Hypochromia. Field. Tomato cytosis is as we mentioned in the red. Under the red cell heading. Quite. This looks like a. What's the, um, platelet level? Deficit level is normal. Normal. Okay. This one appears as hypersegmented. Yeah, you will have one question in the exam that will ask you to list the abnormalities in the blood film. Here, the red cell looks a bit hypochromic. Hypochromia is also a feature. Under the red cell heading. This is a bit tearing. This one and this one. This one we can write. Drop. What's the LFT like? LFT is up to range. The albumin is 93, ALT is 540, and alkaline is 600, which is well. Problem. Then patient has biliary sepsis. All right. Okay. So what is the differential diagnosis based on from what I see? It's probably an MDS. What's, what's the age of the patient? Age of the patient is 50. Okay. I still think MDS. Do not forget UCK it series to comment on from anemia is definitely referral to a consultant. Sorry. Any repeat? Yeah, it's definitely a referral to a consultant. Yeah. So what is your thought? What are you suspecting here? I'm suspecting MDS. MDS. Okay. Okay. This film has now gone to clinical hematologist. What does the clinical hematologist think? I just asked, is, um, what's the monocyte? Monocyte count? The monocyte count there was 1.0. That's normal. Well, slightly elevated. So what does the clinical hematologist think? We have only two clinical hematologists this time. One is Russia, and the other one is Iman. Very nervous gentlemen. Russia and Iman, if you can comment on this blood. Um, regarding the white, you want me to comment on the blood? They report this blood film report. Clinical scientists have referred this blood film to you for a suspected MDS, and the patient has biliary sepsis in ICU, and this is the blood film. I have told you the values as well, the liver function test. Okay. Uh, there is leukocytosis with the left shift. Granulocytic left shift up to myelocytes. Neutrophils, um, are hypogranular with some dysplastic features in the form of pseudo Pelger-Huet anomaly, some, um, hyposegmented nuclear forms. What else? Uh, I think rare hypersegmented. I noticed. Why, why you scroll the photo? Rare hypersegmented. I saw. It's a birth of dysplasia. Anyway, regarding, um, RBCs, there are, yeah, I noticed this also abnormal segmentation of the neutrophils. Let's go to RBC comment. Uh, there is, um, anisocytosis, also includes normocytic, normal chromic, occasional macrocytes, few microcytic hypochromic cells, some stomatocytes also. Um, am I right? I saw occasional NRBCs or it was by mistake. My eyes not occasional. There are a lot of NRBCs we have seen. So I'm right. So I will add the leukopathic picture at the last of the report. But regarding, um, platelets, there is, um, thrombocytopenia. So what to recommend or what to advise? What is the impression? Impression is it goes with the, uh, myelodysplastic syndrome. So I have to assess first level of, um, vitamin B12, LDH, um, to rule out henic deficiency also, iron profile and ferritin level. But ferritin level is deceiving in this patient because you mentioned that he had the sepsis. So iron profile, vitamin B12, LDH, just that maybe we, when the patient is out of infection, we can proceed for bone marrow. Am I right? What is MDS? How do you define MDS? Uh, I got your point. I'm telling this patient has biliary sepsis. The LFT is that is very abnormal. So I will follow from my side. I will follow up this patient has hemolytic picture. Yes, it can be secondary to infection as well. MDS is unexplained cytopenias or abnormality in a single lineage which is more than 10%, but that cytopenia should be persistent. This patient has developed thrombocytopenia because of the infection and liver disease. His liver is very abnormal. It is not secreting any thrombopoietin. Right. He has a lot of NRBCs because the bone marrow is under stress due to sepsis. He has all the lineage of the myeloid. You see this much myeloid or this much band form in the most plastic syndrome. Most plastic syndrome is usually very low counts, but they are persistent, not here. We have a history that patient has biliary sepsis affecting the liver. That's why the patient has stomatocytes. Some of the cells are macrocytes as well because of the liver effect. NRBCs are there because of bone marrow stress. Phagocytes, granulated neutrophils and band forms are there because there is infection. Thrombocytopenia is there because liver is affected and infection leads to thrombocytopenia as well. Myelodysplastic syndrome should have some blasts as well. There is NRBC, myelocyte, dysplastic neutrophil in MDS, but they are persistent and longstanding. Every septic patient that admits to ICU or hematology ward with abnormal platelets does not mean there is MDS. Nowadays, there is a lot of influenza A virus, respiratory syncytial virus. Every second full blood count in the NHS has thrombocytopenia or neutropenia. It does not mean that they have MDS. We will, we will suspect MDS only if it is persistent and longstanding, there is no other cause. This patient, this patient has sepsis, abnormal liver. That's why you are seeing the abnormalities. You expect some toxic granulation. Then yes, but I can't see anything there. What's the CRP like? CRP is 387. Pretty conclusive. No, Dr. Iman, the explanation here is during the infection, the neutrophils can use its granules to fight the infection. So at some level, we can see the neutrophils hypogranulated during the course of infection. It can be no problem. Some of them have granules here, and they are blue dots. Yeah, as well. Yes, yes. I mean, I mean, it is not a MAHA that we have. We have to see toxic granulation. I mean, I expect to see a lot more myelocytes too, to be honest, in severe infection. This patient has quite a lot of myelocytes, and if you can see this death crystal, the blue crystal, that's not good. Pretty much end stage, isn't it? Yes. Severe sepsis usually gives you this death crystals as well. Okay. So this was sepsis. Some people made it a differential of CML last time, which was wrong as well. CML should have basophils and, okay, maybe lost as well. Now, this next slide is again a 56-year-old, and he has developed some skin rashes which is not responding to any cream or antihistamines. So this is power 10. White cell count is 21. Hemoglobin and platelet count is normal. What does the clinical scientist or hematologist? It's about leukocytosis. Try to know. I am on the cells and make the picture clear. Clear. There may be. I would like to make it big for you to see if you have any thought about it. Yeah, that's a blast. Info blast. Maybe. What about the nuclear? Nuclear material being like that? Stained, isn't it? Sorry, how old is the patient? 56. What about this normal? This pro-lymphocyte? This is a lymphocyte. What sort of lymphocyte? I'm interested. You know, what sort of lymphocyte is this? It looks like cells of Sezary syndrome. Cerebriform nucleus. Why do you think this is Sezary? Do you have any features? I couldn't see any Sezary as such. Could see any like, I would say this is the best picture of a. Yeah, there's like certain cleft in the center, isn't it? There are a lot of lines like the, yeah, cerebriform. Well, concentrating on this Sezary, do not forget that there are two Auer bodies as well. You have to report that in the exam, otherwise you will lose marks. Problem as well. They may give you some history about that he has any from a road accident during childhood leading to this. One has a lot of groups, yeah, in the nuclear material. Yes, some. It's not very common, but it is exam favorite. The nuclear material is a bit immature, but there are a lot of grooves, just like the brain, cerebellum. Yeah, it's bad. That's a lot clearer. Searching for other. Yeah, this is a brain but without. So what are the clues of having Sezary syndrome here in the scenario? What doctor? What, what are the clues? Like the skin manifestation is the patient has rashes. Patient will have pruritus or rashes that is not responding to any treatment. In that case, you will be worried whether this patient has any skin lymphoma that is the cause of pruritus in this patient. The nuclear material is a bit mature and not like a typical blast. One. All right. So what phenotype do you expect in Sezary syndrome? Any? Is it CD4 positive, CD8 positive or negative? What do you think? The loss of CD5 and CD7. It is CD7 negative. Negative as well. What else? Or the clonality. Which marker you will check? PCR, gene rearrangement, and T cells. We check TCR gene. Okay. Let us see the, um, it is CD7 negative. It is CD8 negative. 25 negative. And the positive one are the CD4 is positive. 2 and 3 are positive in this patient. You have to exclude the other T cells to get a diagnosis. But mostly depends on clinical features of the patient, which is the unresponsive pruritus or rashes, eczema. Secondly, the specific morphology of the T cells in the blood, which is the cerebriform shape, or you can see a lot of grooves in the nuclear material, not like the L cells of other. And they are CD25 negative. CD25 is positive in T cell and ATL. CD7 negative. CD7 is strongly positive in PPL. They are 56, 57 negative as well. Is a feature of LG here? CD1 negative. CD10 is a feature of among the T cells. So these features will help you in the diagnosis of Sezary syndrome. And for all T cells, the clonality is based on TCR. There is no specific translocations or mutation in Sezary syndrome like other T cell lymphomas. PPL is inversion 14. LGL has STAT mutations, but this one does not happen. So Sezary syndrome comes in the exam very frequently. It is rare in practical life, but common in the exam because it has morphological features. That's what. Now you have seen T cell lymphoma. And let's see other lymphoma to differentiate the lymphoid cells. This is power 10, and you can say that the white cell count is more than 500 because only this one field contains more than 100 cells. Let's go to power. This is peripheral blood of a young boy with high white count and very profuse epistaxis and there are some bruising on the rest of the body as well. What do you see? Numerous blasts there. They are all thrombocytopenic as well. I count for the blues. Looks more like an AML. So you can see the difference between these blasts and the blasts in the previous T cells. They were a bit mature. There were probable nuclei, but they were a bit, the nuclear material was a bit mature and has a lot of grooves. Here, there is perivascular as well. Some of the cells have two nuclei. But the nuclear material is a bit open, and they don't have any grooves. Why would the patient be bleeding? One is thrombocytopenia. Is there any other cause of bleeding with the high white count? We have discussed this multiple times. Clinical hematologist, if your junior asks you why this patient is bleeding, I'm giving him platelet transfusion, but still he is bleeding. Platelet count after transfusion is 70. What's his coagulation profile like? PT is prolonged. Okay. PT is prolonged. So it's a coagulopathy as well then. But what is just 1.1? That should affect both PT and PTT. But this patient has only PT prolonged. Is it muscle be prolonged? 70, that's very long. As you had any, um, um, hemophilia A factor assays done? Yes, one screen was done, which contained factor A1 antigen and reciprocal activity. Okay. What do you want to know in among these three? Some hematologist was expecting you to ask me this question. The colleague from the lab is asking clinical question, which is very impressive. High counts everywhere. High counts lead to acquired von Willebrand disease. If it is high red cell count, if it is high platelet count, if it is high white cell count, it will consume your von Willebrand multimers, leading to acquired deficiency of von Willebrand disease. We have read that in thrombocytosis, we have read that in polycythemia, and we are reading it again and again in AML as well. Even CML patient or CMML patient, if their counts are very high, they can lead to acquired von Willebrand disease because the von Willebrand multimers are absorbed by these cell membranes, and there is deficiency of antigen, leading to bleeding. That's why if you replace platelets in this patient, patient will still bleed because the actual thing is acquired von Willebrand syndrome. How would you manage this patient acutely? Just one answer. This patient is bleeding. White count is 900. What to do to stop the bleeding? Hello? Yes, leukapheresis. We will do leukapheresis to reduce the white cell count because if I give him platelet concentrate, the white cell count is still high, and it will be increasing more and more until I start chemotherapy in this patient. I have to reduce this white cell. With the reduction of the von Willebrand multimers in the patient, it will increase by itself, and the patient will stop bleeding. These are the questions that are asked in the exam, and people fail their exam because they have not answered the supplementary question in the exam. Yes, everyone knows that this is AML, but reporting of the blood film will carry only five marks. There are other five marks as well, which will have such questions like this. Why is the patient bleeding? What is the urgent management? What are the standard risk cytogenetics? What are the adverse risk cytogenetics in this patient? And that's why people will come out from the exam. Yes, I picked that up. This was AML, but when the result comes, they will fail. In practical exams, unfortunately, 0.5 marks matter. People have failed their exam by 0.5 marks as well. Should be very. This slide is for the pathologist and clinical hematologist. This is a 56-year-old patient, um, with eczema and pruritus. This is power 4, and this is the. I want you to pick up the abnormalities and your suspected diagnosis. Can it be hypocellular marrow with reticulin or fibrosis pointing to myelofibrosis? One point. You are thinking about myelofibrosis in the history. I mentioned this patient has recurrent eczema and pruritus. And as well to add more, patient has bony pains. Make it power 10 to show you where is the fibrosis. So you can see the fibers which are in front of you are visible in the background. It is very red due to the presence of a lot of these. This has again fibrosis. If I make it to 50 and then ask you to rethink on your diagnosis. This is power 50, and this is the fibrosis. What do you think? It's all cellular area. MH. This is cellular. And any comments about these cells? These are long, slender, M-shaped. So what are these cells? Nucleus is pushed on. Can it be myelocytes or plasma cells? No, plasma cells are not like that. Um, they are all cylinder shape, elongated or spindle shape. So this is the feature of monocytosis. Monocytes are like this. Oh, yeah. Monocytes. But they said like they are almost like plasma cells, but with the nucleus pushed to one side. Plasma cells have nucleus pushed to one side. And here the other feature is that you can see the trabeculae. They are distorted and thick. We go to call the trabeculae are quite thick and there is a bit of distortion as well. And the other feature of systemic monocytosis is that they have fibrosis around the trabeculae. This is called peritrabecular fibrosis. The body part is okay, but you will see fibrosis in these areas. Like here, this part is fibrosis. Is here, and in this fibrotic area, you will see the mast cells. Another feature of identifying mast cells from the type of slide they give to you in the exam. If they give you any G stain, like this is a G stain, which they usually do not give in the exam. All the, all the slides will be. If they gave you any stain, and it is G stain, you should think about monocytosis because monocytes appear better on G stain. If they are giving you a G stained slide, then your first differential in your mind is that maybe I'm looking at monocytosis. Systemic monocytosis has peritrabecular fibrosis. They will give you fibrotic areas, which will have very trabecular infiltration. They will have infiltration in the peritrabecular area. But here the cells are elongated, spindle shape. In LGL, the cells will not be elongated. They will have infiltration like these small cells. A lot of cells will be. We will see other as mast cells. They appear better on the G stain. If you go to your HMDS or lab and ask them how do you see mast cells in the lab, they will tell you we use G stain for visualization of mast cells. These dotted cells are all systemic monocytosis. Is a cause of myelofibrosis. And these all elongated cells, they are mast cells. These are all mast cells. Elongated. What would you see in the peripheral blood film then? In peripheral blood film, mast cells are very rare. There will be only the cilia. I have one blood film of mast cell leukemia only, which is very, very rare in practical life, and it will not come in the exam. It has few monocytes, round monocytes in the peripheral blood. And in the aspirate, in systemic mastocytosis, if you have bone marrow aspirate, you will see either elongated mast cells or round, very granular, deeply. If I manage to find the aspirate somewhere, I will certainly share with you. But it is the time that usually comes in the exam part two with systemic mastocytosis. So we have, if there is any question, you can ask, otherwise we will meet again. Thanks again. Thank you very much. Welcome. If there is nothing else, then we will meet next Sunday again.