Anyone w/other blood/bone marrow disease with ITP?

Hi,
I was diagnosed with ITP in 2001 and have responded minimally to all treatments, but I have managed to keep my spleen. Rituxin in the one drug that got my platelets a little over 100,000 back in 2007. Now they are hovering between 10 -20,000. I never had a bone marrow biopsy because all other counts were normal and doctors did not feel the need to do one. Recently, I had hemoglobinuria (dark urine) and was then tested for a bone marrow failure disease called PNH (paroxysmal nocturnal hemoglobinuria). Sure enough, it was positive. I then had a BMB and the test for PNH was also done on it and it was positive as well. There is no telling how long I have had PNH, but we would have never done the test for it because I have never had any symptoms, except low platelet count. It is all very complicated now, because I now have ITP and PNH which are both autoimmune diseases of the blood and bone marrow. PNH is an ULTRA rare disease and can cause a low platelet count just by itself without ITP. It causes your stem cells to be produced without the adequate enzymes/proteins in the cell wall that protect them from our immune system. Therefore, they are destroyed when the complement (immune) system is activated. It can also cause dangerous clotting due to the break down of RBC's. The problem is that most people are never tested for it and can take years for diagnosis. My hematologists think that the ITP and PNH are some how related, but it has not been proven. I was just curious if anyone else is facing more than one autoimmune disease of the blood or bone marrow? It has been very scary for me. It seems like just when I have learned to live with ITP and the fear of bleeding, now I have to worry about PNH and the fear of clotting....I know, it doesn't make any sense.

Amber

Amber:

I don't recall anyone here with a PNH diagnosis. There have been several over the years though with ITP and Autoimmune Hemolytic Anemia which is another blood related autoimmune disorder. Some have Evans Syndrome or TTP which are two other ones. There are also some here, including me, who have APS which is a disorder where the blood can clot, even with low platelets. I can understand how you feel having to worry about clotting and bleeding at the same time. Luckily, my ITP has been in remission since I found out about the APS because I now have to take a daily aspirin. Thankfully, I have not had to go on to harsher blood thinners (yet).

The answer is yes, there are a few others who have multiple blood disorders at the same time. Not sure if we can help you with the PNH though.

Thanks for your reply Sandi,
Evans Syndrome is what they thought I originally had, but the hemolysis was intravascular and Autoimmune hemolytic anemia is extravascular. Intravascular causes the hemoglobinuria, which is what I had.
Anyway, it sounds like APS and PNH are sort of similiar. PNH does cause organ damage do to the destruction of RBC's as well as clotting. Some folks with pnh also take blood thinners, but of course, I can't. I really want to try the rituxin again, but I am afraid for my platelets to get too high now! I heard that Rituxin can work even better the 2nd time around for ITP. Didn't you have two rounds of it and the 2nd time you couldn't do all 4 treatments right? When I was first diagnosed with ITP, I read this forum ALOT, but never joined, not sure why. But, I learned so much about ITP on here and it is one of the reasons why I still have my spleen. At least if I could get my platelets under control, then I could just concentrate on the PNH. My hemo is actually more worried about the clotting than he is the bleeding. I may have to go on Soliris which is the only drug available to help with PNH, but it is an IV drug every 2 weeks for the rest of my life....not to mention it is $400,000/yr! I'm trying to avoid it as long as possible Being able to take blood thinners would prolong me having to take it because other than the risk for blood clots, I am only mildy symptomatic from the PNH.

I would also prefer my platelets to be lower than they are. Funny how things change!

You are correct about my Rituxan experience - good memory! That was a long time ago. Sometimes it does work better the second time - I've seen that here a few times.

It is a tough thing to balance, truly. In these situations, some doctors think a splenectomy is the way to go to keep platelets up so you can treat the clotting issues. But there has been a LOT of research lately that shows that a splenectomy can raise the clotting risk; not just because counts might go too high, there are other reasons (can explain if you want me to). Anyway, knowing what I know now, I am also very glad that I kept my spleen. You never know what the future holds.

I hope you can find a balance and treat both successfully. Tough decisions ahead.....

I remembered you because you were one of the reasons why I pushed to have rituxin. My hemo back then wanted to do a splenectomy first and I refused and ask to try rituxin first. Because of the outcome you had with it, I had every right to hope it would help me the same way. Thanks for that!! I only did it once and it lasted for about 2 yrs and then they went below 20,000. Been a rollecoaster ever since.
My hemo now does NOT reccommend a splenectomy for me because of the clotting risks you mentioned. Nor does he reccommend promacta because it has been shown to have clotting risks as well. Ideally, my platelets should be about 50,000. They are 17,000 right now and if they go any lower, I think rituxin will be the next step.

It seems as though you have a very wise hemo. I've come across very few who are aware of the clotting risk. I'm impressed!

How high did your counts get with Rituxan? Do you have a plan in place to treat the PNH if your counts respond? Would your hemo be willing to just go with one or two infusions of Rituxan instead of the full round? Or maybe smaller doses for the four infusions? Maybe you'd get counts up enough, but not too high. I don't know. There have been studies that smaller doses are just as effective as the "normal" dose.

Too bad Rituxan does not treat the PNH (as far as I can tell) or the Soliris does not treat the ITP since they are both monoclonal antibodies. I wonder if there have been any studies on that? They probably work entirely different, but it's an interesting thought.

Do small doses of Prednisone do anything for your counts? Sorry, when I get on a subject, my mind starts spinning with possible solutions. I'm sure you've discussed all of this with your doctor.

Amber, sorry to hear about your condition.

I thought the symptom for PNH is low RBCs rather than platelet.
Was your recent symptom only dark urine?
I thought dark urine(blood in urine)is haematuria.:huh:
I have ITP & long time haematuria. Could it be a sign of PNH?

I hope you can have a good solution to your problem.
Best wishes.

Yes, I love my hemo now. He is VERY familiar with rare blood disorders and is the "expert" here in the Houston area.
Rituxin only increased my platelets to 109,000 (as far as I know). He would be willing to do whatever I wanted to do. He is very good at listening to me and if I only wanted to do 2 infusions, I think he would do it. Although, it's not like they went sky high when I did 4 infusions! I think when and if my platelets go up, we would just monitor the PNH still. I have a D-Dimer test done every month and an LDH level that checks for the amount of hemolysing going on. The higher the LDH, the more hemolysing and the more at risk for clotting. The weird thing is that my hgb is perfectly normal. Normally, people with PNH are VERY anemic and have DARK Brown or black urine frequently. I have neither, so that is what leads him to believe that I don't have much hemolysing going on. Anyway, if things did change, then I would probably start the soliris. It's just a HUGE committment. I don't know if soliris helps ITP, but there have been some studies that have shown that there was an increase in Platelets. Most specialist will say that it does not increase them and most people on the PNH support site have not seen an increase. Some of them have very low platelets as well, but it is due to PNH. Mine is ITP because I have responded to IVIG, Prednisone, and rituxin. Although my intial response was good to Pred and ivig, that soon disappeared. Now if I jump straight to 60-80 mg of pred, they will go up some but not drastically. Not enough for me to live with the side effects. I have had so many and some are neurological/muscular, but they won't say pred caused it. Distiquishing the ITP as a separate disease from PNH was difficult but prednisone and IVIG will not improve a platelet count associated with PNH. Also, the PNH could turn into aplastic anemia which is very common. Boy, wouldn't that be great...Bone marrow can't produce platelets and when they do, the body destroys them! LOL
So, to answer your question, I do NOT respond to low doses of prednisone.
And, who knows if Rituxin helps PNH. Don't think there has ever been a study done on it. Probably "scientifically" it shouldn't, but you never know. I don't think any of the specialists really "know" what goes on with these autoimmune blood/bone marrow disorders!
Sorry about the long post....

Lindy,
Yes PNH most commonly causese anemia, but it has not caused it in me at all. People with PNH can also have low WBC's and Platelets. Not every case is the same. It is a strange disease. I went to the hospital because I had a very dark urine a couple of times and I thought I was having bleeding in my kidneys from ITP. Platelets were 3,000 and I was all ready to go ahead with a spleenectomy and then bam...it was something different...PNH. The dark urine is a sign of PNH because the breakdown of RBC's happens intravascularly and hemoglobin is passed in the urine. So, your urine tests positve for blood but if you closely look at it, there are only a few RBC's. If you have had this, then I would absolutely be tested for PNH. I don't think many people know about this disease because it is ultra rare and only 10,000 in the world have it. I think one who may be diagnosed with "ITP" could have it. There are a few people on the PNH support site who were originally diagnosed with ITP. I think it is because people aren't being tested for it and it is being overlooked. There is a specific blood test called a flow cemetry that tests you for PNH. IT's the only way to tell if you have it.

Thanks Amber for sharing the info.
Was wondering how long did you have ITP before you are diagnosed with PNH?
How is flow cemetry done? Is it just a blood draw like CBC?
Sorry for all the questions.

Lindy,
I had ITP for 9 yrs before

Flow cemetery is done by a lab draw, but there are only certain labs that can run the tests.
If you have any other questions feel free to ask away!!

No, your counts didn't go up too high from Rituxan. Would be worth it to try again. You seem to be very informed about both - good job!

Thanks Amber for the info regarding flow cytometry.

Sandi,
I think it is just a matter of time until I do the rituxin again. If they get near or below 10,000 then I will do it again....just no more prednisone!
I had to educate myself as much as possible on the diseases that I have because I am my best advocate for my own health. If I had just listened to the doctor's, then I defintely would NOT have a spleen today for sure! It also helps me not to worry so much when I really understand what is going on. I will let you know if and when I do have rituxin. I also thought there was another drug in trial right now for ITP, AKR5 01? Is that right? Don't know much about it.

Lindy,
Please keep me informed if you have the blood tests for PNH. It's probably unlikely that you have it, but I would rule it out for sure. Like I said, whe would have never know I had it if I had never had the dark urine. That was my first sign. I wish you well

Amber,
Will check with my doctor in my next appointment.
Thanks for the good wishes.
Hope you get better too.

Btw, your RBC & WBC counts are normal except platelets, what about hemoglobin count?

Wow, I'm getting lots of info here. My hematologist mentioned possible PNH for me about a year ago, but I have no idea whether or not I was ever tested for it. I go tomorrow for an appt. and will ask. It hasn't been mentioned since. I always have hematuria on a microscopic level and have occasionally had an elevated LDH level. Too bad my own hematologist is away on vacation but it might benefit me to see a different one in the same office as they all have their own opinions on things anyway. I respond a bit too well to normal doses of Prednisone and it makes my platelet counts skyrocket, all the way up to 700,000. Then I typically have to do a faster taper than recommended to avoid clotting issues. So, tomorrow I'll either have crashed again or stabilized. The Prednisone has adverse affects on me as well, like driving me crazy. I still have my spleen, thankfully, but have been told it may need to come out on an emergency basis, especially if I crash again.

Kathy Ichter

Lindy,
My HGB is 13.4 which is normal. When you are tested for PNH, it is based on yout WBC clone size to determine how severe your PNH is. My clone size is 70%, which is high. It's just by the grace of God that I don't have more symptoms....

Kathy,
If your LDH is elevated and you have had hemaglobinuria, then you definitely should be tested!
Some of the older tests for PNH are not as accurate as the new one. Please ask your hemo about it.
I am so glad that I can provide useful information for you guys. Hope you are able to get some answers!

Kathy - I'd question the "emergency basis" spleen removal. If you respond to meds, there's no reason for emergency surgery or spleen removal at all.

My hematologist believes that by removing the spleen, there is a chance that my ITP could be cured. I am totally against it; however, he did have me go get the big three vaccines for pneumonia, meningitis, and Haemopholis B Influenza "just in case" my ITP spins out of control and they can't stop the bleeding, and have to remove the spleen. I know there is no cure out there for ITP. I would hate to go through major surgery only to end up in the same boat again, with low platelets plus not have a spleen. This is what I get told every time I have an ITP flare. We discuss it, then put it aside. Sometimes knowing too much can make things more difficult. I dread Prednisone too, but it does work. Everytime I get put on it I have to remind my Dr. to use half the normal dose because my platelets respond a little bit too well and skyrocket up too high.

Oh, the vaccines are standard for spleen removal. If I ever do lose my spleen, my Dr. said I would not be able to fight as well against those illnesses, should I ever contract them. He also warned me about being out in the woods and getting bit by ticks and contracting Lyme disease or Babesiosis, something else contracted from being bit by a tick. Great, I live on the edge of a state forest.....

My own Dr. was not who I saw today (Platelet count 167,000, hurray!) The work issues came up but I forgot about the PNH. Not to worry though, they have me on a pretty tight rein for the next several weeks and will be monitoring me weekly for platelet counts to be sure they don't crash again. I could still have antiplatelet antibodies floating around in my system from my last virus. I have to learn to write things down so I remember the questions to ask when I'm there....anyway, I will ask about testing for PNH, or if I have already been tested. My file is so thick from all the CBC's in it over the past four years.

Kathy

Kathy,
There are other things you can try, like rituxin that aren't as hard on your body and may give you a longer lasting result.
I would try other things before doing a spleenectomy. My platelets have been down to 1000 and I have managed to keep it and I don't respond that well to anything! Rituxin was my last hope and it worked for me

Amber, how are you getting on?
Is your platelet count holding up or you're into Rituxin?
What about your PNH? Hope its stable.
Good luck.

Platelets are down to 11k. Looks like I will be getting rituxin within the next week. Just trying to find a facility that will let caremark specialty pharmacy ship them the rituxin so I can pay a $40 copay instead of $5000 out of pocket.
The PNH is stable, all my other counts are normal (strangely enough). I can't seem to get my potassium up to a normal level. That is starting to concern me a bit.
How are you Lindy?

Amber, good to hear that your PNH is stable.
Is strange but good that your other counts are normal.
Hope you can get rituxin at copay price.

I have been having neck pain lately which is giving me bad headaches.
Trying to fix a physio session soon.
Haven't check with my haemo about PNH yet, only seeing him end of this month.
Will keep you posted.

Amber - I have the same problems with potassium and have for years. I struggle with it constantly. OTC's do not work.

Lindy,
Definitely keep me posted about the PNH. I finally got some answers today about the Rituxin. I found a hematologist/oncologist that is willing to let me have the drug shipped to her office and I will have it infused there. It's gonna cost me $1100 instead of $5000.

Sandi,
What do you do about your potassium? Has anything ever helped you? I had two oranges and a banana today. been eating them like crazy, but it doesn't seem to help. He said he would call me a prescription for supplements...

I take a prescription potassium supplement.
Erica

Erica,
You have issues with potassium too? I wonder how many of us have this issue?? Strange....

I also have problems with B-12 levels, ferritin, and Vitamin D levels. I'm beginning to think I have an absorption problem.

About the potassium, I have it monitored every 3 to 4 months by my Rheumatologist. If it's low, I get prescription potassium. I wish I could just have a standing script like Erica, but my doctor is afraid to do that because high levels can be dangerous. In the meantime, I go though hypokalemia symptoms all the time. I take OTC's daily and eat bananas - doesn't do much.

Hi!

It's been a little while since I've been on this question, but I finally got tested today for PNH. How long does it take to get results back? I was thinking probably a couple of weeks. My Dr. said if that test comes back positive, he will call me himself as PHN is so rare. That would certainly fill in a few gaps and puzzle pieces of my health history. Other than fatigue and low platelets once in a while, I've had no other outstanding symptoms.

My platelet count today was 196,000 and the past two counts have been right at 200,000. I'm in a spontaneous remission, which seems to be the pattern and it's just what I do. I get sick with a virus, my ITP flares up, then a short course of Prednisone treatment, taper off of that, and I seem to stabilize all on my own after that. Now at least I don't have to be a human pincushion for a few months! Does anyone else have track marks in their arms from having all those CBC's done for ITP? I have a couple of good spots to draw blood from in my arms, and a couple in my hands, but that's it. My veins run and hide now when I'm at the heme office!

I'll keep you posted as to what the PNH test results are. I'm sure I'll have a ton of questions for you if it's positive!

Take care!

Kathy

Wow Kathy! Lucky for you, I would love to respond to prednisone like that! I'm on my second rituxin treatment and still only 10,000 platelets
The PNH test only takes maybe a week to get back. I bet it is negative because it is so rare, but it is better to know, especially since you are having symptoms. What did your hemo think when you asked him about it? Did he feel like you had enough symptoms for it to be that?

Should I do another round of rituxin if it only helps a little the first round?