Newest CBC, starting treatment soon?

Hello everyone.

Recently got a CBC after receiving a massive amount of bruises on my legs and two “goose eggs” on my ankle and shin. The results came back and my levels are at 13,000. Yikes. It’s the lowest they’ve ever been, coming up on one year of being diagnosed. My doctor is not in the office until Monday, but the doctor on call told us I may have to start Prednisone, but we would discuss it with my regular doctor when we visited the hospital, likely this Tuesday. If you have any experience with Prednisone, or any other treatment really, please share. I am very new to this “treatment” aspect and though I know it is different for everyone, I would like to hear other people’s stories. Thanks!

Teen
I recommend you read this the latest guidelines
ashpublications.org/bloodadvances/article/3/22/3780/428877/Updated-international-consensus-report-on-the
(I know it looks a broken link but it works I've tried it )
It is lengthy and a bit technical in places but well worth it. It explains all the different investigations and treatments.
I don't know how old you are . There are 3 sections for adults, pregnant ladies and children, so you can skip the middle one.
The more information you know the more involved in your treatment plan you can be.
Don't let them keep you on steroids for long, the side effects aren't worth risking.
Please let us know how you get on.

I have been reading a ton about Prednisone and the long term side effects don't seem like something I want to risk. I have confidence in my doctor that she will do what is best for me but I don't want to stay on the steroids, there are so many things that I don't want to risk and cause more issues. I will update in this post when I go to the hospital, it should be sometime this week because my counts are pretty low and I'm becoming very active again. We were told I may have to start Prednisone by the doctor on call, not my regular hematologist, so my regular hematologist might have a completely different plan, and may want me on another treatment.

Very wise. Avoid steroids if at all possible
I was admitted as an emergency 6 years ago with a count of 12 and covered in huge bruises from head to foot after an accident in the garden. Prednisolone worked well at a high dose but whenever I tapered below 20mg count plummeted so was tried on various other treatments, Azathioprine, MMF & Fostamatinib none of which worked then Romiplostim where counts would swing from 2 to over 200. Eventually went onto Eltrombopag which has been more effective than any of the others in maintaining a count generally between 40-80 which suits me just fine.
I finally tapered off Pred over a 3 year period and took my last tablet on 16th May this year.
I would take it again but only as am emergency rescue for 3 days. I am never ever going back on it long term.
If I were you I would ask to try Eltrombopag or Avatrombopag before anything else, especially with Covid 19 around. You don't really want to be suppressing your immune system.
Have you been tested for H Pylori? If not I would ask to be.

I have heard good things about Eltrombopag, which I think is also known as Promacta? I have a fried who is on it, as well as Prednisone, but her ITP is a lot more serious than mine, she has had a splenectomy and her count is currently 8,000, even with her meds. What is H Pylori? I have never heard about that. I would love to have a count between 40-80. I used to get so upset when they were at 50,000, but I would much rather prefer 50,000 over 13,000. My doctor said something about a bone marrow biopsy before a certain treatment, not completely sure which, but I know there is one that can do something to your bone marrow and she just wanted to make sure it was okay before we started that treatment, if we did it at all. I will try my best to be as involved in my treatment as possible, but I'm still a minor, and will be for a couple more years. I would like to stray away from the steroids, but the ultimate decision is up to my doctor, but I will make sure I have my say in it too.

Teen
H Pylori is a bacterial infection of the gut known to cause ITP. Often people have no symptoms at all so it is worth discussing it with your haemo.

All medications have 2 names, the trade name given by the manufacturer, which can differ between countries, and the generic name which is the name of the chemical. Eltrombopag for instance is called Promacta in the USA but over here in the UK is is known as Revolade. Sometimes I see trade names on here from USA folks and I have to google them to find out what they really are!! In the UK nurses and doctors don't use trade names, medications are always referred to and prescribed by their generic names which is why you will find me using generic names on my posts.

Eltrombopag, Avatrombopag* (Doptelet) and Romiplostim (Nplate) belong to a group of drugs that have no effect on your immune system and are called thrombopoietin receptor agonists [TPORAs]. Basically they fool your bone marrow into thinking it is not making enough platelets so it makes more. They carry a very small risk of causing a condition called bone marrow fibrosis which I really wouldn't worry about at this stage.
Maybe your haemo is thinking of starting you on a TPORA and wants knows what your baseline bone marrow is like and that is why she mentioned a biopsy. I've had one, it is bit uncomfortable but doesn't hurt.

With regard to your haemo making the ultimate decision because you are a minor, over here in the UK a parent or guardian would be the ultimate decision maker until you reached the age of consent which is 16. Do make sure you have your say. It is your body.

*I'm no sure this has been licensed for paediatrics yet.

Ah, I will see if I can get tested for the H Pylori bacteria, though we are almost completely sure I inherited the autoimmune disease trait from my mom, she has Hashimotos, or it was from the Gardasil vaccine, but it is always good to get tested and make sure. I've been reseraching each medicines and I mentioned to my mom that since Prednisone is an immuno-suppressant, I think, I'm not sure if it's something I want to risk, with the virus and everything. I should have rephrased myself, the doctor will decide what to do and my parents will have the final say so. Until I turn 18, over 4 years from now, my parents will have the ultimate say so in signing off on any treatments, but I have made sure to tell my parents that since I'm old enough to comprehend what is going on, I should have a big part in deciding what happens with my treatment, and I believe I have done enough research and am wise enough to know whether or not something is going to potentially hurt me.

Finally going to the hospital to see my hematologist tomorrow! Last draw was about a week ago, but I only went to the labs, but now I will be seeing my doctor. Interested to see the difference within a week. We were told by the doctor on call that we may have to put me on Prednisone, but I'd like to stay away from that if possible. My parents are against the idea of a bone marrow biopsy, recommended before a certain treatment, Promacta I believe can do something to your bone marrow, which I'm pretty sure would require an IVIG before, but I would just like to see higher levels. Any improvement is good for me. I will update after my appointment tomorrow!

Do you know what your count was with last week's draw or will you find that out tomorrow?

It was 13,000 last week. I just left the hospital and my counts were 30,000 today! It could go back down but I’m happy that I saw an improvement. It’s the first time since being diagnosed that they’ve gone up on their own without treatment or an infusion. We have a plan laid out, and I have an infusion scheduled for September. If the infusion does not sustain, we will start steroids while we wait for approval for eltrombopag, we have to do some stuff with my insurance to get it. I’m hopeful for the future!

Good news teen - happy for you! What is the infusion that will be used if needed in September?
Most people do have to get some of the treatments approved by their insurance - I had a WinRho IV back in 2002 or 03 and it had to be approved before I could have it.

Fingers are crossed your count has increased by the time September rolls around!!

I will receive the IVIG again. I got it last October prior to a dental procedure.

Teen
How long do you have to wait for insurance approval?
If you take steroids for more than 3 weeks you will then have to taper off them gradually

Sorry for taking so long to reply, I've been a bit busy, but I'm not completely sure. My doctor didn't specify. All I know is there is no longer a requirement of a bone marrow biopsy to start eltrombopag, but it does have to be approved, though I'm not completely sure the time. I would rather not do steroids at all, but in the end it's my parents who will sign off on all the papers and make the final decision, but I'll make sure to have my say in it.

Teen, it's been a while since we have talked about bone marrow biopsies here on the forum so I'll regurgitate my understanding.

Recall that a ITP diagnoses is a diagnoses of 'exclusion'. That is, they test for a bunch of diseases and they all come back negative. Because there is no direct lab test to see if the immune system is at fault, it is declared the immune system is the cause and one gets the ITP diagnoses.

But in actuality, the diagnosis is not definitive. It's been awhile but I think MDS, the beginnings of bone cancer, and ITP are all still possible. The problem is that one should NOT give Eltrombopag/Promacta/Nplate to someone with a potentiality of bone cancer existing. The drugs could make it much worse. That is why they want a bone marrow biopsy. To officially rule out bone cancer.

The story doesn't end there. Actually, if you've had IVIG and your platelets responded nicely, then that treatment rules out bone cancer and MDS. Having a response to immune system globulin's means it is an immune problem - that the diagnoses really is ITP.

Ack, but the story still doesn't end there. One of the things that Eltrombopag/Promacta/Nplate can do is cause scarring of the bone, aka fibrosis. So many doctors like to have a baseline of what one's bone marrow looks like, in terms of preexisting fibrosis, before starting the drug. For example, suppose there is already some scarring. Then it becomes important to not make it much worse with high doses of the drug over many years.

Have I made sense? Did I get the story right?

Hal, you made complete sense. I had an excellent reaction to IVIG, and autoimmune diseases run in my family, so we were pretty sure it was my immune system going haywire. (Even though I think it was the Gardasil vaccine that wrecked me up).Last summer when I got sick and discovered I had low platelets, I had a ton of draws. We ruled out all of the other stuff and ITP was my official diagnosis. I think they wanted to do a bone marrow biopsy before the certain treatment just to make sure everything was alright, but my hematologist said it was no longer required for that certain treatment. i'm not completely sure though, I don't know much.

Teen
Thrombocytopenia is a diagnosis of exclusion. Sometimes it is secondary to a disease/condition/medication but if no other reasons are known primary ITP is diagnosed. It used to be diagnosed as idiopathic thrombocytopenic purpura (ITP), idiopathic means no recognised cause. In 2007 to idiopathic was changed to immune. For a good description of the reason for name change and ITP in general see this link www.ncbi.nlm.nih.gov/pmc/articles/PMC3410635/

A group of medications called Thrombopoietin Receptor Agonists [TPORAs] such as Romiplostim (N Plate), Eltrombopag (Promacta or Revolade) and Avatrombopag (Doptelet) used to treat ITP carry a small risk of developing bone marrow fibrosis. Last year a study called 'Thrombopoietin Receptor Agonists: Ten Years Later' was published, see this link www.haematologica.org/content/104/6/1112
I have copied the most relevant bits from it regarding bone marrow fibrosis (which is graded MF0-MF3) below

"Early concerns were raised regarding the possible induction of bone marrow fibrosis because of sustained stimulation of megakaryopoiesis .... results of the published trials showing that, in most patients, grade of fibrosis did not change during treatment....while a slight, non-progressive reticulin fibrosis (MF-1) was observed in 10-50% of patients. In one study, a moderate increase in fibrosis (MF-2) was observed in 18% at median time of treatment of 2.5 years,48 whereas in three other studies, fibrosis progressed ..or developed ≥MF-2 during the study periods in less than 10%.... Severe grades of fibrosis (MF-3) were extremely rare in all studies...... In general, it does not seem that TPO-RA induce substantial fibrosis ...... fibrosis regressed in most patients after discontinuation treatment; in a few patients, fibrosis regressed despite continuing therapy.....
There is no consensus for patients on TPO-RA as to whether or how to monitor bone marrow (BM) fibrosis. At the moment, hardly any centers perform routine BM biopsy in TPO-RA treated patients. However, if a biopsy is performed and MF3 or collagen is discovered, then it is recommended that TPO-RA be discontinued. With moderately increased fibrosis, e.g. MF 2, a patient may continue TPO-RA but may need a repeat biopsy in six months. Older age and splenectomy could be associated with higher grades of BM fibrosis; fibrosis was not associated with type, dose or duration of treatment"

I hope you find the links useful. I have been taking taking Eltrombopag for over 3 years and have no signs of fibrosis.

My hematologist said that a bone marrow biopsy was apparently no longer required for eltrombopag, nplate, I’m not sure why, but she said that we would monitor my bloodwork monthly and if there was any sign of abnormality we would discontinue it or do a bone marrow biopsy then. This is a last case scenario, however, she hopes that the IViG in September will boost my levels up and keep them there, though it’s very unlikely. I perform in several performances of The Nutcracker every year, and since I am getting older, I will be getting more advanced roles. (Level 6 out of If I have to be lifted or do any sort of partnering, and the ivig has not kept my levels up, we will do prednisone while we are waiting for eltrombopag approval. There is also a possibility of me going away during the summer to train at a ballet program, which would include partnering and lifting, if the coronavirus is over with, and I would need treatment then. My hematologist seems reluctant to treat me, even though they do get pretty low at times, and it’s tough not being able to do some of things I want to, but some of the side effects of the medicines aren’t exactly pleasant. I’m trying not to worry too much about it, theres nothing I can do but wait and make sure I’m not doing anything that poses a danger to me.

Your haemo sounds very wise regarding Bm biopsies. I hope you get to your ballet training programme. I quite agree with you about medications.Side effects can be worse than the IPT itself as I discovered with Prednisolone, Mycophenolate, Azathioprine and Romiplostim.

Thought I would hop on this thread and give a little update. I received a MASSIVE bruise on my arm, out of no where, and I have no idea how I got it, so I went in to get my levels checked. My little rise didn't last very long. Back down to 19,000. My doctor is once again not recommending treatment, unless we want to do an IViG. Its becoming the same cycle over and over again, and it's quite frustrating.

Teen, I lived with 'easy bruising' for a couple of decades before being diagnosed with ITP. Thought it was just part of getting older. What I understand as easy bruising is like this. If one bumps up (eg with one's arm) against something rigid but there is no pain, without ITP, there is no bruise. But with ITP and low counts this contact does cause a bruise but it didn't 'ouch' at the time. So a bruise can seem to come out of nowhere but actually you just bumped against something and it didn't hurt. That's why one doesn't remember how one got it. There was no ouch'ey event.

Right. Since young folks often spontaneously recover it can be hard to justify treating with harsh drugs. Having said that, it does seem like Promacta is pretty benign.

I don't know how I got it, it must not have hurt when I hit it, but it hurts alot now, even after 2 weeks of having it. It does have a slight bump to it, but I ice it like my doctor says. I'm coming up on one year of being diagnosed, and only ever received one infusion of IViG. I also went to the dentist and found out I have a cavity, but I was given a sealant and prescribed to come back in 3 months. If the cavity develops more, I will have to get an infusion to get the cavity filled. I don't want to do treatment, but it stresses me out to have levels this low and they just keep getting lower. The cycle of low levels going down every month frusterate me, even though I know theres nothing I can do to change it.

The first I had any clue that I had something wrong with me was about 2 months ago when I noticed rather large bruises on my legs. Not a concern at first because on my job Im always throwing myself about and getting knocked about a bit. But these bruises were larger than normal and werent healing quickly. Then about a week after that I began developing the nastiest almost black bruises all over my arms in areas that I would have remembered taking such a heavy blow and then on my upper torso. So unsightly and unusual that one day one of my coworkers said go check that out. This is not to mention the deep fatigue that I had been feeling much worse than usual. Also I noticed that my skin seemed to be more brittle with tiny little cuts on areas of my body in which my clothing was tight. Especially on the tops of my feet because of my shoes. And the day before going to the dr for that first blood test I had developed almost overnight the worst case of Petechiae on my lower legs. I did not know at the time what it was and I certainly had never experienced anything like that or the horrid black blisters on my tongue and on my gums which also appeared almost overnight and vanished just as quickly when my platelet levels rose.

Needless to say I had the dr's visit, they took a cbc, and instead of waiting 5 days for my appointment to discuss they called me later that afternoon and told me that I had to go to the Emergency Room immediately because my platelete level was 2...and so it all began. Im assuming that with me the bruising doest really happen unless im really low like sub 10 levels.

Finally with my level at 262, however long it lasts, im no longer bruising and no Petechiae. Well, there is one large bruise on the shin of my right leg. Im just going to hope I dont remember having hit it.

and allow me to say, as someone who never goes to the dr(until now which will be from now on it seems) and who has only been in the Hospital ER once for serious dehydration and who hasnt spent the night in a hospital since tonsillectomy when I was a kid, the cost of hospital stays(6 days in the cancer ward 4 weeks ago followed by 4 days in the cancer wards ICU 2 and a half weeks ago, the cost is insane. The cost of a single IVIG treatment is tens of thousands of dollars and each treatment of Rituximab is about 35 thousand if I remember correctly. Thank goodness for insurance. A real mind blowing reality check for me

The treatment is very expensive. At the time I received my IViG infusion, we were switching insurance providers, and they billed us without insurance. I believe it was $30,000, for the amount I had. Luckily, we got it sorted out and it was reduced to $3,000. I do not like the treatment, and I know it is costly to my family. My doctor has discussed another IVIG dose if we really want to raise my levels. I mentioned the big bruise on my arm, and while it is normal for bruises to stay on me for a long time, I have had this bruise for a very long time and it has shown no signs of improvement. It still causes me pain when barely rubbing against it, and this is not normal for me. I have been diagnosed for nearly a year, and haven't had a bruise as bad as this one. It is concerning me, and my parents, and we likely will be visiting the hospital this week to talk to my doctor, as the last few times I've gotten my blood drawn, I have just visited the labs. My doctor has told us that since I have likely had it for a long time (my first CBC in 2015 my platelets were in the 90's- did not receive another blood draw until 2019), it won't go away anytime soon. She does not want me to start on any drugs like Prednisone or Promacta, but she says IVIG is always an option if we are feeling extra worried. My parents are going to discuss the option of IViG, and I'll likely visit my doctor sometime this week or the next.

I'm trying to recall if anyone here [now or in the past] has gone into a remission due to IVIg.

How are you doing teen?

I’m doing pretty well. I went in to see my doctor the other day. My levels went back up to 24,000, which is a good improvement from 19,000. We decided to hold off on the IVIG for now, and I have one scheduled for November because I have a cavity that needs to be filled and my dentist will not treat me with 24,000 platelets and my hematologist doesn’t want me doing any dental procedures without levels higher than 50,000, and we know my levels won’t get there without treatment.

Glad you are doing well teen - are you having any symptoms with your count at 24k?
Wouldn't it be nice if your count went up on its own in November so you wouldn't need treatment before your dental procedure?!
Take care now!!

Just the normal little things, small bruises where I've accidentally hit something. No bleeding symptoms, which is great. It would be awesome if my count went up on its own before November, but considering I haven't had counts higher than 50,000 since my diagnosis, besides when I got IVIG, we will likely need to do it in order for me to get my cavity filled. But, I had an excellent reaction to IVIG, and we know it works for me, and if I need it, it will be perfectly timed to last me through my performances of the Nutcracker in December, assuming we get to perform.

Teen
I just came across this www.ijohsjournal.org/article.asp?issn=2231-6027;year=2017;volume=7;issue=1;spage=30;epage=34;aulast=Bansal
It has tables with required platelet counts for dental procedures. You may get away without IVIg