Dr. Camilla Masias

We need to make sure that your platelets are not low for a number of other reasons before calling it ITP. I don’t know if you’ve ever played the game, at the board game Clue, where you start ruling out you know, different suspects for a crime. It’s a similar situation. We need to make sure that you don’t have any liver disease, any bone marrow problems, any infections; any medications that could be contributing to low platelets. And only then can we call it immune thrombocytopenia.

Narrator

Welcome to the PDSA podcast, Bruised but Not Broken: Living with ITP. The diagnosis of a bleeding disorder like immune thrombocytopenia may leave you wondering, how can I really live my life with ITP? PDSA's podcast, Bruised but Not Broken: Living with ITP, brings empowering stories, the latest research and treatment updates, lifestyle tips, and answers to the real life questions the ITP community is asking. Here’s your host for this episode, Barbara Pruitt.

Barbara Pruitt

Hello, and welcome to Bruised but Not Broken, Living with ITP. In today's episode, I'm talking to Dr. Camilla Messias, a hematologist and clinical investigator at Miami Cancer Institute, which is part of the Baptist Health of South Florida. She's also an assistant professor at Florida International University. Her work focuses on platelet disorders like ITP, and she's authored a number of peer-reviewed articles in this area. I'm really looking forward to this conversation as we talk about how ITP is diagnosed, what tests are truly necessary, and how those guidelines can help both physicians and patients make more informed decisions along the way.

So welcome, Dr. Masaias, and thank you so much for joining us. I want to mention that you are on the Clinical Guidelines Committee at the American Society of Hematology, correct?

Dr. Camila Masias

That's correct. Thank you for having me, Barbara.

Barbara Pruitt

I know that's quite an honor. And there's, what, maybe 15 clinicians on that committee? Yes.

Dr. Camila Masias

Yes, yes. About so. We are; I worked on developing the updated guidelines of the original ones that were created in 2019. where you were part of it, of course. And um this time we just focus on very specific questions that we wanted to answer as the field you know evolves in the world of ITP.

Barbara Pruitt

That's great. And these guidelines not only cover what tests need to take place in order to diagnose ITP, but also it covers the treatment of ITP, correct? Correct.

Dr. Camila Masias

Yeah, the updates, well, the guidelines of the revision of the ones that will be published this year essentially answer questions regarding the first line of treatment in patients with ITP. And then we ask the question about what is the best if there's a best second line therapy of ITP. So those were kind of our PICO questions. And then we went along into add remarks on splenectomy. So, we were focused more on treatment mainly because now we have a good problem to have, which is we have a number of alternatives to treat patients with ITP. We just i don't know exactly kind of how do they fit one after the other.

Barbara Pruitt

Right. And I know since the last guidelines were written, like you're saying, there's a lot of alternatives for treatment. A lot of, um, treatments have come to market and have been approved by the FDA that were not included in the previous guidelines. So, it's important that those are now included and that there's an update.

Dr. Camila Masias

Thank you.

Barbara Pruitt

We know that ITP is a diagnosis of exclusion, meaning that there's no single test that tells you that you have ITP. So, what there is, is there's testing that needs to be done to exclude certain things. So, if, if we could go over what those tests are, what, what is actually recommended for that diagnosis, that final diagnosis.

Dr. Camila Masias

Yes, yeah, absolutely. So, you know, ITP is where your immune system mistakenly attacks your platelets, which are, of course, many of our audience will know this already, are the tiny blood cells that help stop bleeding. And what; how we call it the instances of exclusion, it's because we need to make sure that your platelets are not low for a number of other reasons before calling it ITP.

Barbara Pruitt

Right.

Dr. Camila Masias

I don't know if you've ever played the game, at the board game Clue, where you start ruling out you know, different suspects for a crime. So, it's a similar situation. We need to make sure that you don't have any liver disease, any bone marrow problems, any infections; any medications that could be contributing to low platelets. And only then can we call it immune thrombocytopenia purpura.

Barbara Pruitt

Right. And I'm assuming the first sign that you might have something going on is a CBC, correct?

Dr. Camila Masias

That's correct. So, we start with a CBC. Many patients will present with a clinical suspicion of low platelets, meaning they will have tiny red or purple dots in their legs, or maybe they'll have, and maybe they've noticed excessive bruising or other forms of bleeding, while some other patients may just be referred because they went to their PCP and they noticed a low platelet count. And how we know that a patient has a low platelet count is by getting a complete blood count. And it's really kind of like a snap a snapshot of your blood's health. We will get information about your platelet count, but also about your other counts in your blood, including red blood cells and white blood cells.

Barbara Pruitt

Right. And what about the peripheral blood smear? That's done on a little slide, a glass slide, correct?

Dr. Camila Masias

Yes, that's correct. So, kind of going even a step before that. So, when you go to the doctor to get a CBC, you will get your blood drawn on a purple tube. And that purple top tube will have, and that's the one that we use to run a CVC. And it contains a chemical that prevents blood from clotting, so the machine can count your cells accurately. We really need to look at the peripheral blood smear because the machines aren't perfect, and a lot of things; our need the human eye to take a look and make sure that there's nothing else other than a low platelet count. ITP should not have anything, any other abnormality on the peripheral smear. And here, I think we need to differentiate our first suspect in our in when we're ruling out other causes of ITP and it's a condition called pseudothrombocytopenia which is just a fancy name to call platelet clumping so some people in the general population may form clumps on these tube that we use to draw a routine CBC and what that'll do is that the machine will read the platelet count as low

When in fact it's not low, it's just that the platelets are being clumped all together. And how we diagnose that is when we look at the peripheral smear, we looked at the clumps of platelets and we say, Hey, no, this patient has a normal platelet count. It's just that the platelets were all clumped together. And there's nothing wrong with a platelet function. It's just something that happens to some people.

Barbara Pruitt

For some reason there's clump and does, is does that, I know this is another question, but could that lead to clotting issues?

Dr. Camila Masias

No, no, it's just a reaction to the um the substance that we use to prevent the blood from clotting on the purple top tube. So, your doctor may say, hey, you know what, let's just get that CBC again, but this time we're going to use a different tube. So, you're going to get a CBC in a site what is called a citrate tube or a different type of additives to get an accurate count.

Barbara Pruitt

Okay. Okay.

Dr. Camila Masias

And that should resolve the problem. Emphasis on should, because there are still a very small percentage of patients that will have clumping on the second tube. But again, it's more of what we call a laboratory abnormality. Clinically, patients do great.

Barbara Pruitt

Okay. Okay. Okay. And what would another test that you would do to help rule out other diseases or problems?

Dr. Camila Masias

Absolutely. Yeah. Once we start with a CBC and we determine that the patient has what we call isolated thrombocytopenia. When we look at CBC, we want to make sure that the red blood cells and the white blood cells are normal. Patients with ITP should have no abnormalities in their white blood cells. They may be a little bit anemic if they've had some bleeding from the ITP, but really nothing beyond that. So, moving on, once we've determined that, we want to make sure that the patient has not, you know before going into the labs, even documentation of the patient's medications.

It's extremely important because some antibiotics, herbal medicines, antiviral medications, medications used for another different many different types of medical problems can cause a low platelet count. So, we want to make sure we're looking into that. Then we go into infections. We typically, and this is recommended on the ASH guidelines, but it should be routine to get an HIV and hepatitis studies um to make sure that the patient doesn't have these infections contributing to their thrombocytopenia.

Barbara Pruitt

Okay, that makes sense. And in that medications, yeah, sometimes people think medications are only the prescription drugs, but herbal medications and, you know, those type things, vitamins that you can buy without a prescription are also important for you to know.

Dr. Camila Masias

Absolutely, absolutely. And, you know, unfortunately, sometimes we don't even know what is on these herbal remedies we're taking, and it's not uncommon to see those as a culprit for low platelet count.

Barbara Pruitt

Wow. And what about H. pylori? Because I know that's something that I've had tested in the past.

Dr. Camila Masias

Yes, so H. pylori or Helicobacter pylori is a bacteria that is found on the stool of patients. And sometimes we don't really;  the symptoms associated with this bacteria may vary from patients being asymptomatic to patients having gastritis symptoms, heartburn, and it's associated with a low platelet count. So, it is recommended to test for it. And the way we test it is via either could be with a breath test, with a stool sample. But certainly, if you are found to have H. pylori positive, we recommend treating this bacteria and then evaluate the platelet count again.

Barbara Pruitt

Because I understand that, I know I've read studies in the past that in Japan, it's very successful. The treatment for H. pylori has been very successful for people that had thrombocytopenia.

Dr. Camila Masias

Yes, that's correct. In Japan, in some countries in South America, H. pylori is a bacteria that is extremely common because of the diet and the lifestyle. And so, yes, treating it is recommended as a possible cause of low platelet. Yes.

And I'm sorry, I should mention that it's extremely important to verify that the bacteria has actually, the infection has resolved with that course of antibiotics. So, patients are treated for H. pylori and they need to repeat the test and make sure that the bacteria has been eradicated. some but Sometimes patients may need a second course.

Barbara Pruitt

Okay, okay. And I know that there are some, excuse me, some tests that are not generally recommended.

Dr. Camila Masias

Yes.

Barbara Pruitt

And one of them I think people will be thrilled to hear about is, I'll let you say. Okay.

Dr. Camila Masias

A bone marrow biopsy aspirate and biopsy is not recommended for the diagnosis of ITP because really; we don't really need to look into the bone marrow to so know that a patient has ITP. By looking at the peripheral smear and knowing that the shapes and size of the white blood cells are normal, by looking into the different labs, as I said, making sure there's no infections, no medications that could be causing these.

And then we definitely go through other tests that I'll talk about, but certainly a bone marrow, especially in adults that are younger than 60, is not recommended. so um not for the initial treatment, which as you said, patients are very happy. Patients and physicians were also very happy not to not to recommend this test as well.

Barbara Pruitt

Right. Right. Well, I have to say that, you know, talking to other ITP patients and looking on our Facebook blog and stuff, people are very anxious about getting a bone marrow biopsy done. I've had it done. I mean, I've had ITP for 60 plus years and I have had it done. To me, it was not a big deal, but to some people it is a huge deal and I understand that anxiety. But as far as the diagnostic reason for having it done, there's not a reason to do it.

Dr. Camila Masias

Not upfront. I mean, certainly it's a consideration when we are thinking about specific treatments. For example, if we're thinking about doing splenectomy for one reason or the other. We should do a bone marrow biopsy before, but not to diagnose the disease initially. And I agree, you know, when I asked patients, how was your bone marrow biopsy? Thankfully, I usually they say, as you said, it wasn't a big deal, but the anxiety towards the test is real. And I don't blame them, you know, it's a concern and um it's very dependent on the person doing the test, right? So, it's kind of like a hit or miss.

Barbara Pruitt

Right, right. Well, I know that the 2019 guidelines did not have anything in there regarding immunoglobulin levels and testing. So, do the new guidelines have something regarding that?

Dr. Camila Masias

No, because we didn't address that specific question on the updated guidelines, but we you know talked about it and we really couldn't identify any new developments on that. certainly immunoglobulins are proteins that are part of our immune system. So the thought is, well, if ITP is a disorder of the immune system in which it is targeting platelets, shouldn't we make sure that everything else is working fine? And the answer is, you know it's really, there's not an association between a different or an abnormal level of immunoglobulins under diagnosis of ITP. But it is something that will come up as part of your treatment plan or your treatment journey, because we do check immunoglobulins.

For example, some physicians may want to check it before starting a medication like rituximab, or some physicians may want to check it to make sure you don't have other types of immunologic disease, such as a combined variable immunodeficiency. I think that is something that is certainly thought a lot in the pediatric population, but not in adults.

Barbara Pruitt

Okay, the CVID is seen more in pediatrics.

Dr. Camila Masias

Yes, and I would say that I think in the adult population, when we think about diagnosis ITP, a lot of physicians that treat adults won't really order immunoglobulins, but yet I think our pediatric colleagues do this quite often.

Barbara Pruitt

Well, I think it's interesting too that it's a test that you should do before you take rituximab because, and why is that? Is it that the rituximab would not be a good choice or what's the reason for that?

Dr. Camila Masias

We want to make sure that you start your treatment before starting the treatment for with rituximab. Your immunoglobulins are the appropriate levels because if you were to have a low immunoglobulin level, then we don't want to sort of give you more immunosuppression, if that makes sense.

Barbara Pruitt

Right. Yeah, no, that does. That does. um And I've also heard about a Coombs test or a direct antiglobulin test. Could you explain what that's all about?

Dr. Camila Masias

Yes. So, us going back to how we were talking about that ITP is a diagnosis of exclusion, we want to make sure that patients don't have a condition called hemolysis in which we are breaking our red blood cells as much as our platelets. So, there are different um laboratory markers for hemolysis, including LDH and haptoglobin that we will check in patients that have concomitant low platelets and a low hemoglobin.

So, in those patients that have the two things, we want to make sure that they're not hemolyzing. So, we check that and if the and this is something that will be evident as well on the peripheral smear.

The red blood cells that in patients that are hemolyzing will look either in the shape of a perfect circle or they may look in the shape of a red broken red blood cell. So, if that's the case, then we want to make sure that we find out why and that will tell us this is not ITP. Let's find out why patients be hemolysin? So, the Coombs test, what it tells us, to put it simply, is the patient hemolysin because their immune system is also attacking red blood cells or are there other causes of hemolysis? So, we will again draw the patient's blood, look at, put it on a, send it to the lab and they will tell us if they see antibodies against a patient's red blood cells. And if it's positive, um it goes along with the patient having autoimmune hemolytic anemia in which sometimes can present with ITP and that is called Evans syndrome.

Barbara Pruitt

Okay.

Dr. Camila Masias

And then if it's negative, but the patient truly is hemolysin as we are looking at in the smear and we have other markers of hemolysis that we need to look about look for other causes of non-autoimmune hemolysis.

Barbara Pruitt

Okay. Well, that this just brings home the point of looking at the peripheral smear.

Dr. Camila Masias

Absolutely, absolutely.

Barbara Pruitt

Yeah, and I know that's something that we've stressed at our conferences, that you know with the diagnosis of ITP, make sure that your hematologist has the ability to look at a peripheral smear. You know every hematologist should have a microscope and slides in their office and be able to do that.

Dr. Camila Masias

Yes. Yes, and I will tell you plenty of times, I shouldn't say plenty, but we've had cases that patients come and say, I've been told to come to the hospital because my platelet count is really low. And then we'll look at it under the microscope and we say, um sir, I have good news. Your platelet count is not low. It's just there are all lots of clumps. You can go home. But had we not looked at a smear, we would not have known.

Barbara Pruitt

Right, which makes sense. And we have done a podcast on Evans syndrome. So, um you know, when you're so talking about the Coombs test and that type of testing, we have covered some of that in our Evans syndrome podcast. So, what about, I understand that before using anti D or the brand name is WinRow, is there also a test that needs to be done for that?

Dr. Camila Masias

In terms of, we want to make sure that the, we want to look at the patient's blood type. I would say that WinRow, I think that it's not something that we, it's part of, so of if we think about our current arsenal of treatments with ITP, it has gone out of and preference due to a number of side effects with it. But kind of going through, you know, that the things that I should not forget to mention when we're looking at other suspects. So, we talked about infections and medications. And the other thing that I really don't want to left over left is liver disease. So liver disease is a common contributor to low platelets just because of the way the platelets are produced and how the hepatosplenic circulation work. And so we want to include hepatic function panel in our patients that we are diagnosing with ITP. And while this is not, I believe, on the ASH guidelines, we do want to make sure that the patient's coagulation panel is normal, meaning that they have a normal PT and PTT. Because there is a rare type of Von Willerrand disease that may present with low platelets as well, although the patient's clinical presentation will also help us determine that.

Barbara Pruitt

Right, right. And the PT and PTT are measures of clotting, correct? Correct.

Dr. Camila Masias

That's correct. But what's interesting, and this is what I tell my patients, when we think about stopping a clot, stopping a bleeding and making a clot, we can think about it as a recipe to make, you know, some kind of something, some kind of cake. Maybe you, you know, didn't have the full cup of sugar, you just had three quarters. So, the cake may turn out okay because you, you know, you had everything else. You had the flour, the baking powder, everything was in just not enough sugar. That's kind of how I think about a low platelet count. Everything else is working perfectly. That's why we have a number of patients that are walking just fine. They don't even know they have ITP, but others have some kind of excessive bruising, of course, because everything else in their coagulation profile is fine.

So, all this to say that it may sound, I guess counterintuitive to say, well, if my platelets are low, why is my PT and PDT normal? And that's because your other areas of your coagulation profile are working fine, which is all good.

Barbara Pruitt

Because there's like a cascade when it comes to a clot, forming a clot.

Dr. Camila Masias

Exactly.

Barbara Pruitt

It's not just platelets.

Dr. Camila Masias

Yes.

Barbara Pruitt

It's a lot of other things that add to that.

Dr. Camila Masias

Yes. That's right.

Barbara Pruitt

One final question, and I know it's something that we don't think of very frequently, is a splenectomy. So um when we talk about that, are there any tests that would be indicated to look at your spleen for a diagnosis?

Dr. Camila Masias

Absolutely. Yeah. So, we want to make sure that your spleen is normal, meaning patients with ITP should not have splenomegaly. Their spleen should be on a normal size.

Barbara Pruitt

And splenomegaly means enlargement of the spleen, correct?

Dr. Camila Masias

That's correct. So, when we have an enlarged spleen contributing to our thrombocytopenia, to our low platelets, that's usually not ITP, then that means that there's a reason why the spleen got enlarged and that started causing the thrombocytopenia. Yeah, so that's something that we wanna, you know, patients should have a thorough physical exam and measure their liver and spleen size.

Barbara Pruitt

That's when your doctor is pulling up your shirt and pressing on your stomach and telling you to take a deep breath. They're feeling your liver, your spleen, your abdominal organs. So, there is a reason for that. So is there any other type of a test that you can think of that maybe we've overlooked here in our conversation?

Dr. Camila Masias

That's correct. Yeah. I wanted to bring up the; there's a test called antiplatelet antibody testing, and essentially it looks at the antibodies that are attaching to your platelets. So, in theory, it sounds like a perfect test, right? You may say, oh, let's see if I have the antibodies. If I don't have them, then I don't have ITP. But unfortunately, the test is not great at looking at those antibodies. So only 50 to 60% of patients with ITP will have a positive anti-plated antibody testing.

Barbara Pruitt

Okay.

Dr. Camila Masias

So, if you have a negative test, it doesn't mean you don't have ITP. So, this is why it's not recommended.

Barbara Pruitt

Okay. Well, that makes sense. Yeah, because I have heard of that in the past. So, it's not really necessary to test for that then.

Dr. Camila Masias

Yeah, yeah, for sure.

Barbara Pruitt

Yeah. Okay. Well, this has been very interesting and very informative because I know people always are so curious as well. Have they checked for this? Have they checked for that? Are they sure that this is what I have? And I know it's frustrating.

Dr. Camila Masias

They should. No, no, no.  And I think that's the right way to look at it. You want to be thorough and being your best advocate to make sure there's nothing else going on, especially if you have other symptoms.

You know, if you are a woman and you have joint pain, joint swelling on um maybe a rash or you know other symptoms and they're just treating you for ITP, I think it's important when you say, hey, but what about this? It's not really kind of fit into the ITP picture. So i would say that you know we start with talking to our patients, doing like a very thorough history and then going to the lab looking at their smear and it's really important that we do a stepwise and sort of a disciplined approach if you want to call it like that to make sure we're not missing anything

Barbara Pruitt

Right. Which is the right way to approach it; which is great. Well, I can't thank you enough for spending the time with us today. I'm sure that there will be a lot of patients that will be listening to this podcast now and in the future, because these are things that are very important as far as the diagnosis of your ITP. And should the new guidelines be coming out in 2026?

Dr. Camila Masias

Yes, hopefully soon. Yes.

Barbara Pruitt

And I have to say the guideline process is like a four-year process from all of the research that they have to do, the committee has to do, because it's all evidence-based. It's all determined on papers that have been written about the success and that and the discoveries of what needs to be done when it comes to ITP diagnosis and treatment.

Dr. Camila Masias

That is such an important point. And I think that we could even have a podcast about how guidelines are developed because it's, as you said, such an interesting process on not only being very thorough with the evidence and have you know an outstanding methodological team, but also yeah we always have patients in the group that develop the guidelines because we want to make sure that it's ah two things that need to happen. One needs to be written in a way that we can, can be understood by patients. And the second and most important is we want to make sure that we are paying attention to what the patients want us to pay attention to.

Barbara Pruitt

Right.

Dr. Camila Masias

And there's, you know, I think there's been research on that. And sometimes when we use ask physicians, well, when you have patients with ITP, what is the most important thing for you? And then we ask the same to the patients, and the answers are not the same, unfortunately.

Barbara Pruitt

Right. Right. Well, they're not, they're not walking in our shoes, you know, and, and I think the communication is so important when you're discussing treatment plans and stuff with your doctor. It really needs to be a combination of you and your doctor discussing options and coming to a solution of how you're going to move forward. So that's where the guidelines come in to really help. And when they are published, I know that they will be on the PDSA website so that patients will be able to access them easily. And thank you. Thank you. Thank you for joining us today. And I look forward to having you on another podcast in the future.

Dr. Camila Masias

Of course, I would love that. Very nice talking to you.

Narrator

How do you live your life with a bleeding disorder like ITP? From working in the kitchen with knives, to navigating sharp corners in your house, going out to eat in a restaurant, traveling on a plane, attending a sporting event, even dancing at a wedding. ITP patient, Barbara Pruitt, shares her tips and tricks for moving through life with ITP for more than 60 years. Here's her lifestyle lesson for the day.

Barbara Pruitt

Guidelines for the treatment of ITP. We really are fortunate that they exist. Not all doctors are familiar with ITP, so the ability for them to utilize these guidelines is of great benefit to you. At least there are treatments for ITP, and finding out the ones that work best for you is so important. Whatever treatment you're on, you need to make sure that you take it as directed. Don't ever stop taking it without your doctor's instruction because you could crash. Your platelets could drop dramatically, which could put you in a very vicarious situation. We know that is true with steroids. That is why you are weaned off of them slowly. But it also applies to other medications. Don't stop the medication without your doctor's instructions to do so. And don't ever get in the situation where you run out of your medication. That can be just as dangerous. I know refills, pharmacies, and insurance bureaucracy can be a hassle. But you need to stay on top of it. Let the pharmacy know you need a refill at least a week in advance before you run out. Follow up with them daily. If there's an insurance issue, find out what it is and intervene. If a new prescription is needed from your doctor, call them and ask for them to send it to your pharmacy. If your insurance needs a pre-approval, make sure your doctor has received it and follows up on it. And you need to make sure that your doctor sends it in. If there's ever a holdup, ask questions so that you better understand what is happening. I take notes. I write down the date, the time, who I spoke to, and what they told me. This is really helpful when it comes to dealing with insurance companies and If you get stuck, you can always ask to speak to their supervisor.

If you understand the procedures involved with filling your prescriptions, it will help you get over any hurdles that you may encounter. Be persistent. And don't, I repeat, don't run out of your medication and don't stop taking your medication. without your doctor's instructions. Well, that's it for today. I hope you'll join me next time on Bruised but Not Broken, Living with ITP. Until then, I'm wishing you lots of happy, healthy platelets.

Narrator

Thanks for listening to the PDSA podcast, Bruised but Not Broken, Living with ITP. Made possible by our presenting sponsors, Amgen and Sanofi. Special thanks to Gus Mayorga for composing our theme music. To see what's coming up, visit our website at pdsa.org and subscribe wherever you get your podcasts. Please share this podcast through social media with anyone who you think might benefit from the information and stories we share with the ITP and other platelet disorders community. As always, please speak with a healthcare care professional before making any treatment decisions, but know that pdsa.org is a wealth of information and resources to help you navigate life with ITP and other platelet disorders. Remember, you are not alone.