Transcription
Right, so this is the first case. This is a 58-year-old man who presented to the emergency department with severe shortness of breath, fatigue, and chest discomfort. The biomedical scientist called you because you are the hematology registrar. The blood film shows some abnormal cells, and he thinks that the patient has acute leukemia. Is there any volunteer who can go through the slide? Anyone?
The first one on the list is F. Uh, Comy. Hello. Hello. Can you hear me? Yes, I can hear you now. Um, I mean, in the film, um, I will start from, usually I start from platelets. Um, so this is the 10x film. I will go through the film and then I will make it 50x. Yeah, so that you can see the film. Take a minute, and then you can start reporting the blood. Now, 50x. Yes. So now you can start your report. I think there is some thrombocytopenia. Um, this anemia with red cell anisocytosis and poikilocytosis. So always start filming reporting the film from the dominant cell. What are the dominant cells that you are seeing here? Red cells and white cells. The white cells are dominant here. You are seeing a lot of white cells. Yes. These cells, can you comment on them? It's got a high. Can we bring it a little bit closer? Yes. Okay. So this film has almost everything that I want to ask you. The small, they are, uh, small lymphocytes, and I think they are abnormal. Large cells with high N:C ratio. There are some, some neutrophils are also there, like one neutrophil. The band form. The band form. So these are small mature lymphocytes, lymphocytes which are not common to the normal blood film. So they are here for a reason. Secondly, this blood film has different types of red cells. You can see this red cell has no whitening in the center. This is a spherocyte. Spherocyte. Yes. This is bluish in color and bigger in size. Polychromasia. These are polychromatic cells. Polychromatic macrocytes. This cell has Howell-Jolly inside it as well. This is a band form. This is a metamyelocyte with kidney dip inside. This looks like a nucleated red cell. Nucleated. But it's difficult to differentiate because it is, I think, smashed during this, during the specimen preparation.
All right. So you didn't ask me what is the full blood count of the patient. Yes. What is the full blood count? What is the white cell count? So the white cell count is 24. Okay. The hemoglobin is 7.8. Platelet count is 160. It is normal. Okay. This patient is 58. He has shortness of breath, and these are the counts, and this is the blood. Do you have to say something to your biomedical scientist colleague? He may ask you, "Doctor, can you please go through this screen with me?" Yeah. So, spite anemia, polychromasia, yes. Macrocytosis. What does this indicate? Hemolysis. This patient is having hemolysis. Yes. And the film is also telling you a reason of hemolysis as well, which are these small mature. So do you know why these mature lymphocytes are present? I don't know. I mean, mature lymphocytes, hemolysis, what does that indicate?
All right. So, um, in general practice and for part two exam, if you receive this blood film, you would say the blood film shows severe anemia and leukocytosis. The red, the white cells are small in size and having mature nuclear material. The red cells has, um, anisocytosis showing multiple polychromatic macrocytes, spherocytes, and, uh, NRBCs. Any other NRBC? Um, while going through the film, we will add NRBC as well. The leukocytes contain some, um, high count of neutrophils and, um, metamyelocytes as well. This patient may have an infection as well. That's why I'm commenting on the neutrophils here. The blood film, the platelet count, the platelets look normal. The blood film is consistent with severe hemolysis secondary to a possible lymphoproliferative disease. So the third part of the reporting of the film will be, this patient needs, um, hemolytic screen and, uh, flow cytometry or urgent flow cytometry to exclude lymphoproliferative disease. So now you have reported the blood film. You have commented on the cells, on the findings that you have noted. You have given your, um, probable diagnosis, and you have suggested a plan on the film as well. Right.
So what is the immediate management of this patient? Transfusion. What, what type of transfusion? You do this patient has hemolysis, and we need to, that I mean, DAT test to be sure like there's an autoimmune process ongoing. So that may take, that come back as positive, and it may take 4 hours because you have to request blood from the, uh, NHSBT reference lab. And this patient has shortness of breath and chest discomfort, and he's severely anemic. So what is the, if this patient is in your practice, so what is the emergency management of severe hemolytic anemia? What transfusion? So what type of blood transfusion? Blood transfusion. Okay. So I need a complete answer of what type of blood transfusion you would give to this patient if the DAT is positive and it is taking time. No. Okay. So ideally, we give them Kell, Rh, and K matched blood. I8, um, cross-matched by, uh, cross-matched by I8 at 37° Celsius. But this patient, that is positive, and it will take time, 4 to 6 hours for the blood to be arranged. So we can give them an emergency product. So we will give them O positive blood or O negative blood. O negative. Why O negative? Why not O positive? Universal donor. Negative. So you can, so according to the NHSBT guidelines for a male patient, you can give O positive blood as well to save the O negative blood for childbearing age potential female who are, uh, who are having Rh negative group. I webinar. Okay. Then, then the second component is that because you suspect because DAT is positive, you will give this blood along with a cover of methylprednisolone and IVIG as well. Plus, this patient needs folic acid replacement and iron. This is your immediate management of a hemolyzing patient. All right. And the last question would be, this patient has been diagnosed as a case of, um, hemolytic anemia, warm autoimmune hemolytic anemia. What is the long-term management of this patient? Uh, right. 1 milligram per kg prednisolone. Mhm. And along with the steroid, what do you want to give to this patient? At, um, calcium supplement. BP. So, as said, so the first line treatment for autoimmune hemolytic anemia is steroids. So, for 3 weeks and then start tapering. If the patient responds to that, that is fine. If the patient does not respond to that, then you start tapering and think about a second line of, um, management of the patient. But with steroid, you have to give gastric protection in the form of PPI, bone protection in the form of alendronate. You need to give him folic acid as well because he is hemolyzing and you are trying to keep the hemolysis under control with the steroid. If he fails, then the second line agent for, um, autoimmune hemolytic anemia patient is rituximab. In primary cases, we do bone marrow biopsy before starting rituximab. But in this case, this particular case has some, some small mature lymphocytes in the, um, in the film. So in the beginning, we mentioned that we have sent blood for the flow, and if the flow comes back as CL, then you would think about starting the treatment for CL as well. The autoimmune hemolytic anemia can be primary and can be secondary. If there is a secondary cause present, you have to treat the secondary. Like this particular patient has CL, and the causes of autoimmune hemolytic anemia, secondary autoimmune hemolytic anemia, you should know from the guidelines are many. It can be a hematological cancer or solid organ cancer. It can be an infection like, um, Mycoplasma infection, EBV, CMV. It can be an immune disease like SLE, rheumatoid arthritis, etcetera, as well. And sometimes it is post-transplant. You would have seen autoimmune hemolytic anemia in your post-transplant patient as well, where we give them rituximab to avoid that. So autoimmune hemolytic anemia is an important question for both part one and part two, and it is quite common in daily practice as well. And you should know the pathway which is given in the BSH guidelines. What to do if you are suspecting a hemolytic anemia? So go through that algorithm. It is very nicely explained, and it will help you in management. All right, sir. Can you repeat the source from which we can see the algorithm? It is in the BSH guidelines. Um, they have given a very nice, uh, algorithm where you can find out, um, what to do if you are suspecting hemolytic anemia and investigations in a patient with autoimmune hemolytic anemia. And these are the, the differential diagnosis of hemolytic anemia, diagnostic approach to hemolytic anemia, causes of autoimmune hemolytic anemia, warm primary, secondary, cold primary, secondary, mixed type. This is all given in the BSH guideline, diagnosis and management of primary autoimmune. There is another guideline for the treatment. I think there is another flowchart for the treatment as well. Yes, yes, there are two guidelines, autoimmune hemolytic anemia in the DS. Okay, so that was the first case. Okay.
So this is the second case. This is a 26-year-old female who presented to the emergency department with severe body pains, especially in the, uh, thigh area. On questioning, she just arrived to the UK 2 days back. She's originally from Nigeria, and she says that she has sickle cell disease, but she does not have any card for sickle cell disease or any, uh, blood transfusion card for sickle cell disease. She is 16 weeks pregnant as well, and she looks very pale and in pain as well. So this is the blood film. The biomedical scientist is not aware of the history because the blood sample just says unwell, and he called you, and he thinks that this patient has thalassemia because he has noticed a lot of NRBC in the blood. So the first question would be, report the blood. Is there any volunteer to go ahead? The next on the list is Masra Abdullah. Okay. Next one is Mu Mo on the list. Right. Omar Alri. Sir, pardon me, please repeat your question. This is a 26-year-old immigrant to the UK from Nigeria. She arrived two days back. She presented to the emergency department with severe pain, body aches, especially in the thigh areas, and she is looking very pale as well. She is 16 weeks pregnant. She says that she has sickle cell disease, but she does not have any record with her. The biomedical scientist called you that this patient looks like to have thalassemia because the patient has a lot of NRBC in the blood. You have been asked to report the blood. You can go ahead. Omar. Sir, is there any history of, um, uh, fever, recent fever, or any drug history? There is no drug history, but her temperature is 38.5. Sir, could it, I can see a lot of spherocytes. So, so, so it means I would phrase my question in the form of part two, please write five points from this blood film. Sir, first of all, there are, many, uh, spherocytes and, um, we can see many spherocytes also polychromasia. Mhm. And, um, polychromasia, spherocytes as well as NRBC. The picture is also having few target cells in there. And, um, um, it appears to me as a dimorphic picture. Some cells are small, the other cells are large, maybe that is due to polychromasia or reticulocytosis. And, um, um, maybe she is a compound heterozygous for beta, sickle beta, because we can see target cells as well as microcytes. Sickle cells are also being seen. I can see three sickle cells in this view. How you have seen sickle cells? You have seen target cells, Howell-Jolly bodies, NRBCs, spherocytes. You have seen polychromatic macrocytes as well. Yes, sir. Crisis. Okay. So in the exam, they can ask you, write five points from this blood film or six points from this film. Usually, because the reporting carries, um, six marks. Okay. Now, report this film as you would do in your normal life or for the exam purpose. Sir, I will report it as anisocytosis with the microcytes, 2 plus. Sorry, sir, I have to mute because my kids are talking around. Anyone else can jump in? Hello? Yes. Yeah, there is anisocytosis, there is polychromasia on the RBCs, and many NRBCs can be seen, and there are few sickle cells are also present. And, um, um, in other morphology section, there is the, um, sorry, the target cells are also present. Howell-Jollys are present, and, uh, as well as the WBC count is, the RBC count is low, looks like. And, uh, the WBC count and morphology is fine. I think. Can you please scroll up? There are few, um, neutrophils were present. So here is the, so morphology is looks normal, and, uh, there is another, yeah, WBC count and morphology looks normal, and, um, the platelet count is also so adequate. Anything else I missed? What is your impression? Normally, with this picture, like there are target cells and there is some mitosis, hypochromia, there is there could be some, um, hemoglobinopathies. In that, the, so I should jump on say that either it's thalassemia or sickle cell? Can I just conclude in the form of [Music] hemoglobinopathy? Sickle cell hemoglobinopathies or sickle cell crises? Sorry. So you would report the blood film as this blood film contains, um, multiple NRBCs, sickle cells, target cells, Howell-Jolly bodies, spherocytes, polychromatic microcytes. The neutrophils are hypergranulated. The platelet counts, uh, looks normal. The blood film is most likely consistent with sickle cell crisis secondary to an infection. The Howell-Jolly bodies, the presence of Howell-Jolly bodies and target cells represent either hyposplenism or hyposplenic, depending on the history of the patient. Then you would give your suggestion that this patient needs urgent hemoglobin electrophoresis to find out the percentage of sickle cell and cultures, which is the most likely cause of precipitating sickle cell crisis. So this will complete your report for the exam, and also in daily life, you do like that. We first mention the findings which is present in the blood film, then give our impression, what is the most likely impression, and then we suggest to them what to do next. Right.
What is the management of this patient? Sir, management would include, uh, hydration and, uh, removal of the precipitating factor. If the patient is febrile, give antipyretics, and for infection, give antibiotics, and, um, maintain, uh, his hydration status, and, uh, transfusion. Uh, for transfusion, would, would see if his hemoglobin is, there is the trigger for transfusion, that is more than 9 g per deciliter, we will go for transfusion. Okay. So you are managing the patient's sickle cell crisis by IV hydration, IV antibiotics, thromboprophylaxis, and you are discussing transfusion with the patient as well. Your hemoglobin electrophoresis came out shows to you that the HbS percentage is 80%, and the patient did not respond to, uh, top of transfusion. The pain has gone worse, and she has a patch in the chest X-ray, which is that the patient has pneumonia, because of which the patient has sickle cell crisis. Patient is not responding to pain, patient is not responding to exchange, sorry, to top of transfusion. What to do next? We would go for exchange transfusion. And, um, if she's having, um, sorry, she's having a patch in her chest for pneumonia, we will, uh, give IV antibiotics and oxygen. We will check the oxygen saturation and maintain in the patient on oxygen as well. Okay. All right. This was the second question. The last question would be, this patient has come out of the, um, crisis, and she says that she usually gets four or five sickle cell pain crises in a year. She is not on any management at the moment for sickle cell disease. What is the long-term plan for this patient? Like, this patient is going home now after settling the sickle crisis. Would you discuss with the patient anything to prevent sickle cell crisis in the future? Sir, for prevention, we would, um, counsel her to avoid the precipitating factors, avoid high altitude, maintain good hygiene, and give her prophylactic antibiotics to prevent any infections, and also, um, with sickle cell, they can get recurrent splenic infarcts which lead to autosplenectomy. So we would give her vaccination for meningococcal and pneumococcal and H. influenza infections. Okay. What to do to prevent crisis again? Hydroxyurea. Can you start in that? If you write hydroxyurea in this particular patient, you will fail the question. If you write hydroxyurea in this patient, you will fail the question. Do you know why? Okay. Sir, because she is pregnant. So we can use interferon alpha. Yeah, she's 16 weeks pregnant. Okay. And I haven't learned about the pregnancy. Sorry. Oh, okay. So either we usually hear in our therapeutic transfusion for such patients. So prophylactic antibiotics, prophylactic folic acid, and, uh, referral of the patient to hematology obstetrics clinic. They will follow the patient through the pregnancy, give him, give her a sickle cell card, give her a card of any antibody if she has, give her the leaflet of sickle cell disease, and exchange transfusion if you are thinking about exchange transfusion because if this patient turns out to be having a twin pregnancy and she would be a candidate for exchange transfusion, or if she has any comorbidity from the sickle cell disease, then you would consider her for exchange for and CNS contacts as well, and register her with the hemoglobin of the services of your, um, of your sickle cell. So always consider the scenario for your exam purpose. You can be tricked in any way. Like yesterday, I was telling the part one candidates, do not write general answers. Always address the patient that is given to you in the scenario. This patient was pregnant. Giving hydroxyurea is not a good idea. Fine. And, um, if this patient comes up in the ward as well, part two, so we have new guidelines for the hyposplenic patient. Read about it before going for the exam. Is it a BCS guideline? For the BSH guideline, if you are preparing for UKFC part, you have to follow BSH guidelines or NHSBT guidelines. Okay. All right.
So this is the third case. This is a 92-year-old lady who is admitted in your hospital with urosepsis. The full blood counts, the white cell count is 64, hemoglobin is 98, and the platelet count is 120. The biomedical scientist called you that I have a new chronic myeloid leukemia. So any volunteer to go ahead? Anyone? Mash Yamin is the next on the list. Are you on the list? Ran on the list? S.A. Yeah. So here is the increased WBC count. So I, I just was trying to find out the base of I right now. So there are WBC count is, and there are the myeloid and the metamyelocytes in the same on the film, and few large looking large. Yes, I'm making the film to 50x so that you can see the ratio. Large. The high ratio. And okay, so this is the 50x power. Yeah. So there are few large cells with the high N:C ratio with the open chromatin as well, and few monocytes as well. Few what, sorry? Monocytes. It's on the, on the left on the side up. Is this a monocyte? No, this is not. You can scroll up. It's a monoblast. This is a bilobed blast with typical nuclei inside it. This is another blast. Blast. Here is the blast. It could be in the blast crisis as well. Okay. Can you find the basophils here? Just trying to find some basophil. Why you want to find basophils? I'm going towards CML. Why you want to go towards CML? It consists mostly of blasts. It looks like leukemia, acute leukemia. More of the cells constitute of blasts. Yes, there are a lot of blasts. There are myeloid blasts, there are monoblasts. Yes, myelomonocytic features are seen. Some are myeloblasts, and some are monoblasts. I can't see any basophils so far. That was the opinion of your biomedical scientist colleague. So what is your thinking about that? How will you report this? There are two cells with, uh, blast-like cells with a high N:C ratio, like myeloblast, open chromatin, and as said, that monoblast as well. One is here. These are all blasts. Okay. So how will you report the blood film? Leukocytosis with predominance of blast cells, or is then the comment will be acute leukemia, and we will suggest flow cytometry and bone marrow biopsy for workup of this patient? Okay. So this completes your, um, blood film report. Very good. You have noticed leukocytosis which are mostly blasts, and most likely consistent with acute leukemia, and the special need urgent flow cytometry and invasive investigation like.
Now, the next question is, what is the minimum genetic test that all new AML patients need according to the BSH WHO guideline 2022 or BSH guidelines 2022? What genetic test you need for any new acute leukemia? Karyotyping. Karyotyping is one. And mutational analysis of the, like, um, NPM1 mutations and FLT3. Yes, this is two. PML-RAR alpha, and, uh, mutation. You need either FISH or PCR for inversion 16 or 8;21 or KMT2A. So one is the FISH category or PCR category. Second one is karyotyping. Third one is molecular studies for FLT3 and NPM1. And the fourth one is NGS panel. So these are the four requirements that any, uh, new acute myeloid leukemia patient should have. Sir, what four categories? One is FISH. The other one is, uh, PCR. NGS. FISH/PCR for inversion 16, 8;21, and KMT2A. This is one category. Second category is karyotyping. Third category is molecular studies for FLT3 and NPM1. And fourth category is NGS. Fourth category is NGS. Whole genome. Genome sequencing. Includes it is a part of NGS. Okay. Whole genome is also part of NGS. Yes. Usually, you will take the skin biopsy from the patient and send it to the lab for whole genome sequencing. So these are all blasts. They have high N:C ratio, open chromatin, some of them have nucleoli. So there are two types of blasts here. One is myeloid blast because they have granules in their cytoplasm, and another one is the, um, monoblast. Their cytoplasm is a bit clearer than the myeloid blast. Right. Sir, can we say for in description of a monoblast, can we say that they have ground glass cytoplasm? I read it somewhere. Is it a good term to explain it or no? No. Okay, sir. You have found out that these are blasts, monoblasts, or myeloid blasts, or blasts is enough because now we are not using FAB classification in the UK. We use a new WHO 2022 classification which has made the things easy. This is acute leukemia. Karyotyping will tell us what is the, uh, translocation this patient had. 9;11 translocation is which type of category? Intermediate or what, sir? Intermediate category. Yes. KMT2A, or previously called MLL rearrangements. Yes. Before 2022, it was MLL. Right.
So the last question for this slide will be, what is the management of the patient? Just tell me the bullet points. How will you manage this? [Music] So, for the management, we will from the diagnosis, either I'll go towards just stratification for that patient. That is that comes in the intermediate. And what is the age of that patient? 92. 92. So, for that patient, basically for all these patients in the old age, we assess for the presence of any frailty and then we go towards the geriatric assessment for that patient. And in geriatric assessment, we go for any comorbidity, any polypharmacy, any social support, quality of life, all these things we go to find out. And, and we will also go for the prophylactic antibiotics for that, for that patient. In in such old age, and then we will see either these patients are for the intensive chemo or not. So I think from that, the nice approach was for the V-CLAD and the, and the cytarabine for that patient who were not fit for the intensive induction, as I remembered. And there is another protocol as the platelet count in that patient is very high. So I don't, so as a cytarabine monotherapy will be good for that because normally use for 20 to 30. Sorry for the background noise. 20 to 30%. Um, if the blast count is 20 to 30%, then we'll go for the as a monotherapy. And so the cytoreduction will go for the hydroxycarbamide and along with all the supportive care for for that. So this patient is 92, and at the age of 92, um, the patient usually have multiple comorbidities and they are very frail, and no one would do, um, intensive chemotherapy for them or invasive investigation. Right. So the management steps would be breaking bad news, explain the diagnosis, assess the patient for Charleston Comorbidity Index (CCI), and give her the information regarding the, uh, acute leukemia MDT discussion, and the result would be that this patient is not fit for intensive or any chemotherapy at the age of 92. You cannot do bone marrow biopsy. You cannot give anyone, um, V-CLAD as a cytarabine or intensive therapy. Such patients are for palliative care and hydroxycarbamide. You can give for a palliative purpose to reduce the count. Usually, this is the individual decision to give hydroxycarbamide for palliative care. In some patients, we give, in some patients, we don't give. But if she is, we will not give on the oral. Even on the, no, no, no. She is 92. She must have multiple guidelines. There was, if the patient is more than 75, we can go for that options. If, but I'm considering him more than 75, so I just, just mention these options. You have to look at the patient's condition as well. V-CLAD is a drug which has the potential to cause tumor lysis syndrome, and has a lot of side effects. It is an intensive drug. So she is not fit for any intensive therapy or V-CLAD, and the blast count is more than 30%. So she's not for cytarabine as well. Yeah. If she is very frail, she's not fit for any chemotherapy, she'll be fit for palliative care. But the management steps would be to explain the diagnosis, breaking bad news, give information to the patient, explain the, um, because of the comorbidities and frailty, you are not fit for the chemotherapy. The risks associated with that are very high. It's better to go for palliative care. Discuss your case in the MDT. Present your plan. Everyone would agree with that. No one does intensive therapy or biopsy in a 92-year-old patient, and then inform the GP and arrange discharge for the patient, which will be either home care or nursing home. All right. So I, I'm mentioning the age here. I deliberately mentioned the age 92 just to give you an idea that in the exam, it is your patient which you have to manage, not in general. If somebody says that, okay, I will give, um, due to this patient of black, the judgment is wrong. So how can you give intensive chemotherapy? Don't you think it depends on the frailty of the patient? If the patient is, like, either if it's 92, but if the patient is not frail, then can we go for these options? In that case, or in either case, at 92, if the patient is not, yeah, if you find any healthy patient at the age of 92, you can give chemotherapy. Yeah, yeah. I mean to say that it all depends on our scoring or all these things, and then he will decide that I, yeah, even there may be a patient of 30 years, but he may, he may be very frail. He can have a lot of comorbidities. Even in such cases, even you cannot give intensive. Like we have one 41-year-old relapsed AML patient, we palliated him because he has cardiomyopathy, renal failure, hepatopathy, osteitis. In such patients, we cannot give them anything. But my my theory is that in the exam, we can just give the options for that according to because we will not do all the scoring there, and we'll just mention there. The you will not do any scoring over there. Yeah. So that's why we will give the options for the, um, for the venetoclax and the cytarabine or the palliative care. So there are options according to their frailty for the patient. Just my only query is that, yeah, you will mention that you will assess the patient for comorbidities and fitness for any type of chemotherapy. But I feel that this patient at the age of 92 would have a lot of comorbidities making him unfit for any sort of chemotherapy. But it needs, it depends on my assessment. Okay. Thank you.
Okay, so we'll make this case as our last case. What is the age of this patient who presented with? What is the age of this patient who to find you're showing us? Yeah. So this is a 60-year-old patient referred to you by GP because there is pancytopenia and the patient has abdominal discomfort. The pan-cytopenia was running for many months. So you decided to do a bone marrow biopsy for this patient. So this is the 4x to go through the length and texture of the bone marrow biopsy. The fine, I mean, so it is a good length to find with many trabecular spaces. Yeah. All right. Now, 10x. All right. Anyone want to come in? There seems to be some infiltration. And first of all, the megakaryocytes, the cellularity, sir, the cellularity is good. However, megakaryocytes are reduced. I can't appreciate megakaryocytes much. Is it hypocellular or good cellularity? It's a normal cellular. Patient is 60. It's fine for him. There are many. Yeah. What is this? The one where your pointer is. No, I removed this pointer. In general, sir, in general, is it some, uh, hairy cell like the cells are present in spaces and the cytoplasm around them is shrunken? Okay. So why do you say it is hairy? Can you explain again? Sir, I felt some cells are present in in spaces with their cytoplasm shrunken and, but no, in that particular area, I felt it like that. Is there any meds for that? Meds in that to find like. Okay, right. What is this? Do you think this is TR? This is a nucleus, and this is the whole white cytoplasm, and these are the boundaries of cytoplasm. Yes, sir. And do you think I felt it fried egg like? Do you think it goes with the scenario? Yes. In the scenario, there was pancytopenia on peripheral, on CBC. You told that the patient is 60 years old, having pancytopenia, and this is his finding. So this is the 100x. Oh, sir, what area? Sir, still I will go with this, with what I initially thought. I think it is hairy cell. Yes, this is hairy cell. What I'm showing you. Leukemia. Fried egg appearance. Fried egg appearance is there. Yes, sir. I will go back to 50x because on 100x, look very sensitive. Sir, also at low power, I could appreciate some fibrosis because some cells at the periphery were stretched out. So this finding is packed with hairy cells. The patient, the patient has pancytopenia and he has abdominal discomfort, which is because of the spleen. Yeah. So this is the typical appearance of hairy cell. Let's go to another section and let's see. So this section has has some cells, nucleus with cytoplasm, clear cytoplasm, just like a fried egg. This one, this one. This section also has a lot of. What is the expected flow cytometry? Sir, CD11c would be positive. CD103, CD123 will be positive, and Annexin A1 will be positive, and CD11c will also be positive. 11c is positive. What else? 103 positive. 123 positive. 123 positive. Yes, sir. And, uh, Annexin A1 positive. Oh, on immunohistochemistry, CD25 also positive. Yeah. So the hairy cell panel is 11c, 25, 103, and 123. 123. They are positive. What is the molecular mutation? Yeah, so it is BRAF V600E. Okay. And would you treat this patient? So this section has a lot of, uh, hairy cells. Right. So would you treat this patient? Yes. Why? If he is asymptomatic, then I will not treat. So because he is symptomatic with abdominal pain, that's why you want to treat it. What is the first line drug? Purine analog for that. Okay. Yeah. Um, we usually use cladribine over pentostatin because cladribine is only for 5 days, that's why our choice. Right. So that patient, the patient, if only pancytopenia, then you need to monitor the patient. But if the pancytopenia is persistent, then then you have to consider the treatment. Is there any time period like for the six months you will see for the cytopenia? Is it rapid? No, no. Six months. Six months is quite a lot. If the patient remains pancytopenic for a month or two months, because we usually repeat the bloods every week, then we decide to treat the patient. So if there is a descent in the counts, then we will start. Yes, because if the patient remains pancytopenic for this much long time, then I'm afraid that he may get infection and that can go to sepsis. So a month or two is enough for observation. We will go for the splenectomy for that patient as well because the spleen is symptomatic and abdominal pain. Patient, if it's, no, no, not to me. If he receives chemotherapy and he responds to cladribine, then the spleen will regress in time with that. Okay. But if the bone marrow infiltration, I think, is low, then we will go for that option for that because this, this bone marrow was quietly infiltrated. So we will not think even for the splenectomy for that patient. Nor we say if the bone marrow involvement is low, then then we'll go for splenectomy. Is that true? We we don't do splenectomy for hairy cell leukemia or any other disease here because now chemotherapies are available. Hairy cell leukemia responds to chemotherapy very well, and after responding, you will see the fibrosis will go down in the bone marrow and the spleen size will regress as well. Regress. Okay. Uh, sir, I have a question that when do we add BRAF inhibitor for hairy cell treatment? Do refractory or in relapse patients when you don't have any other options, then you use BRAF inhibitor as a trial. They are not yet proved here in India. I think there is some, there is some time period. If the complete response is like complete remission is more than two years, then even with the relapse, we do not use the BRAF inhibitor. But if the relapse is less than two years, then we will think about using BRAF inhibitors. Even even your complete remission is more than two years, then we will treat with the same one and repeat the same one. Not we will go for BRAF. Is that true? Yes. So if the patient has responded to cladribine and he relapses after two years, then will use plus, uh, plus, uh, rituximab. You will add rituximab. If the patient relapses within two years, then if you have used cladribine first, then you will use pentostatin as a second line drug. But if the patient is refractory or relapses after that, then you don't have any choice of cladribine and pentostatin, then you have to use a trial drug which is more of or inter. What about the anti-CD22? Is that approved? No, no. It has a very, very serious effect of capillary leak syndrome. Still, because that was in the approval time from 2020, and I don't know the current status for that. We don't use it in our hospital even. Thank you.
This is the last slide. Quick one. This is a 29-year-old patient who presented to the emergency department because of bleeding from the gum. So who is the volunteer? Sir, in this slide, on the low power view, I can see the white blood cells are increased, and if you show me the high power view, I will be able to appreciate if they are blasts or not. Okay. Uh, yeah. Um, sir, this is a case of acute leukemia with the with predominantly blasts. And, sir, in red blood cells, I appreciated two schistocytes in the previous, um, previous portion of the slide. These, the blasts are medium to large in size and have irregular nuclear contour, and I can see Auer rods, and some of the blasts have folded nuclei. And, uh, sir, what is this cell with the, um, this, is this we can say, bilobed nucleus or we can say figure of eight appearance? The one it was. Okay. By, sir, this is a case of acute myeloid leukemia because, um, I had, I could appreciate an Auer rod. Most of the blasts are monoblasts because of the cytoplasm is abundant. The slide is actually blurry. It's okay. Yeah. It's okay now. So here you, I can see the cells with figure of eight appearance, the one on the left and the one on the right. They are like those of APML. Plus, okay. It's again blurry. Yes, it's clear now. Thank you. And, uh, yeah, there I can see Auer rods as well in the cytoplasm. Sir, is it acute promyelocytic leukemia? So this is a blast and it has nucleoli as well. This is a blast and it has nucleoli here as well. All right. How will you report this blood film? Sir, I would report this as this peripheral smear showed the presence of high white blood cell count, uh, which are predominantly blasts, and most of the blasts have a morphology of medium to large size, with the folded or bilobed nuclear nuclear contour, and some of the blasts show Auer rods, and, um, and, uh, also in one view, I saw two schistocytes or fragmented red blood cells, and platelet count seems to be reduced on the smear. The features are consistent with the finding of acute promyelocytic leukemia, and, uh, urgent, urgent, uh, what we say, urgent FISH for PML retinoic acid receptor alpha is recommended, and patient should be started on ATRA. Right. So reporting is correct. The blood film shows leukocytosis. The, uh, majority of the white cells are blast cells, or you can say granular blasts, because APML can be granular type and agranular type. Platelets are low, and red cells, and no other red cell pathology. The blood film is consistent with acute leukemia, most likely acute promyelocytic leukemia. This is a hematological emergency, needs urgent admission, intervention like ATRA, and blood for FISH PML RARA, or if it is working hours and you are working in AI hospital, you can send blood for PML stain as well, which will give you the result in one hour. Right. Is this a high risk or low risk APML? Sir, um, um, what do you mean by high risk or low risk APML? So high, the count is more than, if the count, I think, is more than 10, and WBC and platelet count is less than 40, then it will become high. The white cell count is more than 10. This is high, and the platelet count is also included in the stratification, like 40 is the limit, or I'm just confusing. If someone else, it is another risk stratification criteria which is called Sanz risk stratification, which includes white cell count and platelet count, but we do not use it there. We only use the white cell count. High risk or low risk. More than 10, high risk. Less than 10, low risk. Low risk. What is the importance of high risk and low risk? Because if the patient is low risk, then we will, uh, we will, we will not consider it for the chemotherapy as well. We will just treat it with the ATRA. But if the patient is higher, if the count is, uh, raised, then we will just stratify the patient, either it's fit for chemo, and if the patient is fit for chemo, then you'll start with the ATRA as well. And if the patient is not fit, then we'll give the ATRA low dose. So if, if the patient is of high risk, then we consider idarubicin plus prophylaxis for differentiation syndrome. We add prophylactic steroids to prevent differentiation syndrome in the future. If it is a low risk, then it is only the ATRA. Right. Okay. So what blood tests you would like to send for this patient? And why? This is the last question. We will go for the coagulation screen for for the review of that patient. We will monitor about the coagulation screen for that patient. Yeah. So you have sent blood either for PML RARA FISH, which will give you result in one hour, or FISH, which will give you result in 6 hours. And then you need to send coagulation screen. Mention close fibrinogen because the normal fibrinogen testing that is routinely done is derived fibrinogen here, but you have to mention coagulation screen including cross-fibrinogen. Close. Yeah. This patient's platelet count is low, he was having bleed from the mouth, and there were schistocytes in the red blood film as well, which means that this patient may be in DIC. H. Yeah, because I was also concerned about the RBC because that that looks squishy on the smear as well, because that's not the normal one on the. Yes. Then you have to keep the platelet count above 50. You have to keep the PT normal. You have to keep fibrinogen above 1.5, and twice daily full blood count and coagulation screen including PT, PTT, and fibrinogen. All right. Okay. Thank you. Thank you so much. Thank you for the question. Thank you, Doctor. It's always amazing to learn from you. Thank you so much. So last few bits, you these sessions are free, but if you want to donate, you can donate on these accounts. It's up to you. And this recording will be uploaded on my channel. Please subscribe to the channel and share it with your colleagues, and see you next week. Thank you. Thank you. Thank you, sir. Thank you. Thank you very much. Really an interesting and educative session. Like.