#11 Splenomegaly

Before we get into this episode, just to mention, always wash your hands, always gain consent, and aim to ensure that a patient's welfare, safety and comfort is maintained throughout the exam. This podcast is supported by Past Test. Past Test is an excellent resource which has a vast library of photographs, videos and notes which you can use to help prepare for the exam. For ice-based candidates, you can also claim back the cost of the resource using the training support scheme. Welcome back to MRTPI bedside. Today we have Dr. Claire Kelly here with us. Claire, do you mind just introducing yourself and then explaining what your job is at the moment? Yes, so I'm a certain Claire. I'm a hematology SPR in my fun year of the scheme. This year I'm in research, so I'm a clinical research fellow here in the National Regulation Centre, starting an MD with Michelle Allen. Thanks so much for coming on today to talk to us about Splenamegley. As everyone knows, it's quite a common case in the short cases and associated conditions can certainly come up in the long cases as well. Claire, do you mind talking to us through some of the clinical features that you'd be looking for on an exam in such a case? Yeah, so we'll probably take this in context of your MRCPO examination. So I suppose ideally we'd normally start off with examination of the hands. I suppose some features that might be seen with those rheumatoid arthritis, if we're thinking in conjunction of a splen, and then as you move up to the head, then kind of seeing does the patient look likely might be anemic, etc. And if they have generalized palar, I suppose the main thing you'd always want to examine is make sure examine every lymph node area. So that goes through some mental, your submandibular to your pre-regular post-regular to your super-regular and your auxiliary bilaterally as well. And they're really important before you even proceed with your abdominal examination, because if you have lymph nodes, presence, it'll definitely change your differential diagnosis. Then obviously when we go in with the examination of the abdomen, you'll examine all nine of your quadrants, and then it should be usually pretty apparent if there's a splen, if there's a massive splen present with that basic percussion, or palpation apologies. And then with examination of the abdomen or how do you examine first splen, you also start off in the right iliac fossa. It's very important that as the patient breathes in on deep inspiration, that that's when you're feeling for the spleen, so I'll see it moves down on deep inspiration. And there should always usually be a palpable notch in massive splenamegly. And then as I said, make sure definitely that you move your hand then as the patient begins to expire. Whilst you're there as well, once you're examining it's also very important that you maybe look at the inguinal areas, and for lymph node not to use well, just make sure you do that maybe in a civilized manner, because again it's quite close to the patient's brain region as well, just to bear that in mind. I guess for that as well, for inguinal, you could also ask the examiner or turn to the patient and say, I'd like to also examine the inguinal area and they might be appropriate. Maybe appropriate just to mention us. Yeah, yeah, I totally agree with that. So once you kind of feel a mass in the abdomen, the main differential really in the MOTCPI is this a kidney versus this a spleen. That's usually what you think. And then the left upper region. Yeah. So how do we differentiate those so you cannot get above a spleen, but you can definitely get above a kidney. As I said, your spleen will have your notch. Your spleen will move towards the right iliac fossa on deep inspiration where your kidney will not. And your spleen is definitely not belotable as well. Yeah. And that's certainly a question that can be asked afterwards. If you've told the examiner that you suspect there's a large spleen of megal, they might ask you, how do you know it's not a kidney, not a large polycystic kidney. As we've gone through there, there are a few findings peripherally, but the major finding is going to be in the abdomen. So in the abdominal case, it just want to emphasize again that you try and work through your hands and peripheral examination, eyes and face relatively quickly to get to the abdomen and make sure that you spend time there and feel the spleen and make sure that there's not a liver there either. So with our examination, we suspect this patient has a large spleen. How do you sort of approach a differential diagnosis in such a patient? Yeah. So there's a few is breakdown spleen amygdly. I suppose for the purpose of your exam, I might just break it down into if there's an isolated spleen or if there's a spleen and lymph nodes, because that's kind of how you're going to get in your exam. So if someone has an isolated spleen only, and particularly if it's giant, which tends to be in the exams as well, my main differential is there, the chronic myeloid leukemia, CML, and then my other major differential will be mylofobrosis. So there'd be my first two for a massive isolated spleen. If it's kind of a moderate spleen or a mild spleen amygdly, then that kind of brings in the other differentials, which are if you have an EBV infection, C and V infection, you can get like a spleen with it as well. You can get like feltes in a associated rheumatoid arthritis. You can all sketch out your infiltrative condition, such as your amyloidosis and your gochers as well. And what's useful to know is that there are the more common causes in your developed country. So things like lishemises, malaria, etc, which are a little bit more common outside of Ireland, we do get cases here and people who have gone on foreign travel. So just bear in mind that it's unlikely because they're usually quite sick that they'll be there in your exam, but it's worthwhile knowing about as a differential. And then I suppose how would you take it if you had very obvious, maybe heptomegly and lymphadenopthene associated in which it's plenomegly. That thing kind of brings in literally any formal informa. So you can have your Hodgkins and non-Hodgkins or we prefer to break them down into high grade, non-high grade. So your Hodgkins and former, if you start to be a cellar, it's very more common types, flicular informa. They're the three men that we eat, they're very commonly and then I suppose the other one that you should bear in mind is chronically infecidic leukemia. Okay. So there'd be my broad differentials. And what Claire has done there is she's broken them up into different categories. So you've talked through lymphomas and do you've mentioned infiltrative and then infective, which can be a way to certainly learn them for your own understanding and go through them like that. What we'll do is we'll also post maybe a link or a copy and paste a list of a way to learn them in the show notes below. So you there in the exam, you've presented your physical findings and your differential diagnosis. How would you proceed then to investigate this patient? So as hematologist, we most definitely love a fold block count. It is extremely informative and don't ever forget a blood film. So if I take into context a CML, they typically have a very high white cell count and a lot of kind of neutrophils, myelocytes, menomilocytes, or the occasional blasts. If I take in the formation of a myelophilosis again, they can have like a associated tromsodipenia. Again, they tend to get myelocytes, menomilocytes, and occasional blasts as well. The blood film is extremely informative. So don't forget that it's such a basic, cheap, key investigation. I thought you would love a fold biochemistry profile. In particular, your LFTs and the association with the spleen, we like to have renal profiles, just in case tumor lysis syndrome might be up there as a possibility, along with a fold kind of electrolyte profile. For anyone with an lymphoma, LDH, uric acid, serum, protein, electrophysus, and again, we always love to do HEPB, HEPCs, EBV, CMV, and HIV as well. So we've talked through a lot of the bloods there, a lot of the important blood tests. What other investigations would you proceed to do? Based on kind of the clinical history and the FBC, the blood findings, et cetera, it might kind of break down your next step. So if someone has a lymphoma, or you think it's a highly suspicious lymphoma, you'll obviously have to biopsy lymph node to confirm a tissue diagnosis. We prefer lymph nodes over a biopsy in the spleen, the spleen is very fast culture. And then your other thing is that you need to do formal imaging in the form of usually a PET CT, and if that's not available, you usually CT to our X-habitement and pelvis. The other key investigation, then if you're considering something like a myeloproprosis or a CML, is obviously a bone marrow ulcer, and triphine. And you can do various investigations on the ulcer, you can send it for immunofrenetiping, otherwise known as fluositometry. And then we can also do a full carrier type on the patient as well. And so that'll be looking to see if there are 46 X-X-X-X-X-Y, but we tend to get deletions and translocations associated with our various disorders. Maybe a little bit too high tech. I'm giving Claire the eyes. She's broken up perfectly there, though, you've got your bloods, and don't forget to mention your blood film, and be able to have a brief justification for why you do each of these tests followed by either biopsy of your radiology as well, which can include chest X-rays, looking for a bilateral hyaluronic lymphadenopathy. You could also do an ultrasound with a liver in the spleen as well, and then your further CT. Oh, and did we also mention likely not in a cohort in the Irish MRCPI exams, but a lot main cause of the Spendermagley worldwide it would be malaria. So important to mention. Malaria, you supposed to see me at all to be worth mentioning there as well. Yeah. Okay. From Larry, you'd be doing thick and thin. Thick and thin blood for them for your malaria. Thick and thin blood films for malaria. I guess obviously the treatment of such a patient depends on the cause and I had isolated Spendermagley in my exam and I was, by the time we talked through everything, we didn't have time for management specifically but we might just talk briefly about CML and mylofibrosis today. So if just in a brief explanation of what CML is and then what investigations would lead you to suspected diagnosis of CML. So I said CML chronic myloid leukemia so that defines that it's a myloid disorder and leukemia I always think it literally means too many white cells. So how do we treat this? So I suppose the main things that we do for treatment as I said is that the Philadelphia chromosome is your T922 translocation, common MRIPI question in the clinic exams as well as the MCQs. It's worth worth while knowing that. And I suppose it's quite unique in that we can target it that T922 translocation which are drug options. So these are the tyrosine kinase inhibitors. The oldest one that we have is a matnip and we have some second and third generation drugs now called desatinip, phonatinip and boseydunip for reference. So that would be your main first line treatment in a patient with CML. Okay, would you mind just talking us through what they might be expected to know about mylofibrosis? Yeah, so mylofibrosis, again I tried to break it down in my head. So fibrosis means scarring, so scarring of the bull marrow. So that means your bull marrow doesn't function properly and then your spleen actually gets bigger as it tries to make your blood cells. It's actually medullary hematopoicis. So it's kind of nice to know the background of it. So the treatment of it actually at the moment will depend if the patient is asymptomatic or symptomatic. Thrasium-tomatic is just a watching way. If they have symptoms like they have low HBs, low platelets counts, they have weight loss or they have very bad symptoms from their mass of spleen, then we can go in with like a jack two inhibitor called roxylotoninip. So that's actually quite an unexpected. So that's roxylotoninip. Roxylotoninip, okay. It's tough when jacavi is its drug trade name which you don't like to use, but it's just a little bit easier to remember. There is hydroxycarbomide or hydroxyurea as well, it can be used if they have a very high white cell count just to bring it down in the initial setting as well. And the main thing about mylofibrosis is that there's a 30% risk of transformation to AML. Okay. And these patients can become transfusion dependent as well. It definitely can become transfusion dependent. Yeah. So we have we have a lot of our cohort potentially coming into the day ward on a weekly or twice weekly basis to have their bloods and platelets. And Claire, just while I have you here is our hematology and spleen etology expert. Do you mind just talking us through some of the spleen related immune disorders? Yeah. So I suppose you can get autoimmune hemolish minimia and ITP in association with a mild splenamegaly. That's probably to increase the function of the spleen as it's clearing the red cells and the platelets. I suppose for autoimmune hemolish minimia the key investigations again would be your foot block count, your liver profile to check your billy ruban, do a direct and an indirect billy. So I always say if it's come, if it's directly from the liver, that's fine, if it's indirect, it's in the blood and that's my issue. And we do a particular side count to see if they're making lots of baby red cells in response to the anemia. And then an LDH is key, tends to be elevated and then one of the great tests then will be your death screen direct and to lovely test, which will either should be positive in autoimmune hemolish minimia. And then as well as that we normally do a seer important letter free cysts. And then I think that's the main investigations we do. Habtoglobins are diagnostic, they're low. In autoimmune hemolish minimia, the path you will have to go up and detect three to four days to come around. So it's a bit late by the time you have the result. And then I suppose the treatment of autoimmune hemolish minimia then is usually straightforward. You give prednisolone 1 milligram per kilogram until you've turned off the hemalysis, then with a very slow taper on like a weekly basis. So the 10-tugane steroids for over three to four months. So very important you educate on the side effect of steroid therapy, that's a really nice question that leads in for more immune hemolish minimia. Then I suppose it ITP kind of similar in that it's platelet destruction instead of red cell destruction. So again, the 10 to get a very low platelet count. They're very impressive, they don't bleed that much, considering they can come in with platelet counts less than 10. But things to look for out there is that they'll have the little ptiki on the legs and maybe mucosal bleeding or ptiki on the met this one. Again treatment there, first line treatment is more than 1 milligram per kilogram up front of prednisolone as well. I don't think you'd be asking which but second and third line manager does them. That'd be a tricky question and you're doing very well if you've got to that stage. And say some of the conditions where you have hypersplenism related to increased function. Do they sometimes remove the spleen? Do they sometimes do a spleenectomy? We can, that would every first line. And that will be one of our functions is that we would remove the spleen. And as I said, that leads quite nicely as to what we do in preparation of an elective removal of a spleen, which is then kind of similar to what you do after you've removed a spleen in an emergency situation. So again, as a spleen is so important when it comes to the removement of encapsulated organisms that you need to vaccinate people up front. And for all of your encapsulated organisms, so you'll do like your meningococcal, your hip vaccines, etc. And then as well as that the patients will need to be cancelled, that they need to remain on penicillin porphylaxis for a life once you've removed the spleen. So typically we go with calvapen 300 or 23 milligrams BD. Aside from that indication for a spleenectomy, are there any, are there any other important indications that the candidates probably should know at an MRCPI level? For removing a spleen. So those main ones that will be will be trauma because it's all highly vascular that they can get quite nasty hemorrhaging from their spleen. That's really kind of trauma if it's ruptured or said. Oh, very rarely do we get like a rupture secondary to like a lymphoma that's extremely rare, but that can happen. Claire, I think you've gone above and beyond. Thank you so much for talking through everything related to spleen today. I'll let her go. Claire, before I do that, I was going to ask you a couple brief questions. Do you remember what you got in your MRCPI cases? I actually definitely got a spleen. I had a patient with MS on my neurological exam. I had a acromegaly station. I also had a trifrost group re-gurge and. Try cuspid re-gurge. Yeah. And I'm now forgetting the last case. Okay. Not giving me my age, but it was a few years ago. Do you know what's, do you remember where it was? And it's sliger. Sliger, okay, definitely. Yeah. So they definitely have an acromegaly patient there? Can I ask you any advice you'd give to people preparing for their exams? Anything that you remember, any golden nuggets that you remember? I think it's the sense about what you study. There are very high-yield topics that you should make sure you know 100% in a night so you should know all of your murmurs. Like you should know your spleen amegalies, you should know your acromegaly, you should know your renal transplant. It's really good if you have a body and even if you examine, you need to look slick to examinations. I always think you kind of need to go back to your final med kind of going. So mature your very slick to examinations, spend time again on the war. It's on focus examinations of no one clinical findings. And then I forget tutorials on the trigger items that you don't like. There's usually friendly espiores in every hospital. There are very friendly tutorials. Or interviews for podcasts as well. So Claire, thank you so much. We'll let you go. A big thank you again to stepping on Lego who provide our amazing show music. If you'd like to get in touch, they can be contacted at [email protected] or through social media. And if you want to contact us, you can get in touch via our Instagram @mrcpibedsite. Twitter account or ex-account @mrcpibedsite or email which is [email protected]. Thanks again and good luck. Good luck.