Is anyone here considered refractory for ITP?

Hello everyone. I have been a member of this site a while ago (about 3 years ago) and left this site because at that time I was informed I did not have ITP but something else. So time passes and now my new hematologist sent me to the research doctor in my state (Arizona) and he made the following statement.

“Most doctors considered ITP to be refractory if it does not respond to steroids. I considered it refractory if it does not respond to Rituxan.”

So I am back on the ITP bus again and am wondering has anyone else been diagnosed as being refractory for ITP?

My past treatments have been ineffective – steroids and IVIG, or almost lethal – Win Rho. So I am now working with my hematologist locally to get aboard this rituxan treatment plan. Looking for advice and not really looking forward to rituxan, but I guess that is the delimiter I need to pass if I am considered refractory. Of course what happens then...who known.

PS – I am very attached to my spleen and the doctors have told me to not have it removed. I have had whatever bleeding disorder this is (ITP or something) since age 5, so now at 41 this is really getting sucky.

AzThumper

To answer your question, has anyone else been diagnosed as being refractory for ITP?
Even the experts seem to be confused about what refractory means in ITP.

Dr Bussel discusses the term here: (sorry it's a PDF and I can't copy it)
cancer.cwru.edu/calendar/articles/bussel.pdf

The journal "Blood" has this to say: Refractory patients should fulfill 2 criteria. First, they should have failed splenectomy or have relapsed thereafter. Second, they should either exhibit severe ITP (see Table 1) or have a risk of bleeding that in the opinion of the attending physician requires therapy.
link: bloodjournal.hematologylibrary.org/content/113/11/2386 .

I haven't had my spleen removed, so I guess I'm not refractory, and never will be.

Anyways,whatever I am, taking Rituxan gave me 4.5 years of remission. Good enough for me to try it again.

Hope you find something that works. I remember reading your posts at the "old discussions". What did the dr's decide you had?

Hey Thumper - there you are! Believe it or not, I think about you!

I wouldn't consider you refractory until you have failed ALL treatments. Just failing one or two does not make a person refractory. Have you tried the new TPO's? If not, then you are not refractory yet. Plus, you still have your spleen.

I was first told it was a genetic issue, but that was never really explored in depth, as everyone still is on the ITP bus. So at this point with new insurance and on disability, the journey starts again. At this point I am still awaiting the rituxan treatment and then see what is going to happen next.

AzThumper

I've had the Retuxin 4 times and my platelets dropped instead of going up. Retuxin did not work for me. I had 4.5 months of Predisone, which worked at first, but I could not tolerate the high doses for long. I've had the IVIG several times. It does great the first week or two, but drops low within 4 to 6 weeks. So far, I am still waiting for the I.T.P. to go into remission and platelets to hold.

Thank you for sharing. Steroids do nothing for me regardless of the dosage for my platelets. IVIG did very little for me, and what good it did was gone within hours. Let me clarify I went up to a count of 90 to only crash down to 40. I have had WinRHo to only go into anaphylasitc shock and get a wonderful count of zero platelets for four days in a row. So at this point a new set of doctors are now going to hopefully put me on Rituxan to see if that helps at all.

Now at my age, over 40, I was also informed that removing my spleen was not really that helpful and all the doctors agree on keeping it inside of me. What the best guess is that my platelets are just to fragile and shatter. These little pieces are keeping me from bleeding at times, but are not that helpful in most cases. So do I have ITP, maybe, do I have something else, they do not want to approach that until the Rituxan does or does not work.

I had to ask here as I was hoping someone had something I could ask my doctors about. Unfortunately my doctors at this point are more finger pointers and seem to be more clueless than helpful. I understand that having ITP since a child and still having it, never going into a normal range at all regardless of treatment is sort of a small percentage, especially for a male. So I was hoping at some point that something was discovered or learned about ITP that would help. I thank everyone for your support and reading my posts, and I shall keep you apprized if anything new happens.

AzThumper

I am considered to have refractory ITP and had my spleen removed 9 weeks ago (it did not work, counts stayed at 1,000). I have had steroids, win-rho, rituxan and nothing worked. Finally I was given NPlates and after the 4th dose my body started to respond. My understanding is that NPlates have only been FDA approved for about a year.

The only problem with them is that by the 5th dose my body responded too well and my platelets went as high as 1 million and I was then at a very high risk of a stroke or blood clot. I have not had a treatment in three weeks and my platelets have come down considerably I am at 196,000.

NPlates are more convenient than Rituxan, it is a shot that is administered in the doctors office. The side effects are similar to Rituxan, I experience a headache for a couple of days after I receive the shot.

I am considered chronic, refractory, ITP. I diagnosed at 14 (1987) and responded well to steroids until... I had my spleen removed in 1999. Since then I have been up and down, and have failed steroids, rituxan, win-rho, n-plate, and promacta. I was healthy (relative to me) from 2007 - January of this year, when my counts dropped to 2k. I am currently doing the chemotherapy (Cyclophosphamide).

In any case, the worst side effects, for me, have been with the IViG treatments. I receive those about every 2 weeks, or whenever my count falls below 10k. No side effects with N-Plate or Promacta.

I've only been on the chemo for a few days, but I've been pretty lethargic.

I thank everyone once again for your continued information. I have already had one treatment of Rituxan and it simply lowered my blood pressure, but no other side effects that I can feel. I go for my first blood count on Thursday and go for another treatment on Friday. So far so good, as in no adverse reactions that I can tell. I am looking forward to the counts to see if those budged at all. I wish everyone the best with their battle with ITP.

AzThumper

It can take a few weeks, Thumper, so don't expect too much too soon. Good luck!

Good Luck! I know what it is like waiting to hear if your numbers have budged, I go every Wednesday.