Refractory ITP Not Responding to Treatment

Hi, I am a 40 year old male looking for information from anyone that has been where I am in treatment and achieved a full or partial remission.

I was diagnosed with ITP on March 3 2012 with 5,000 platelets. I spent a week in the hospital on 110 mg Prednisone/day and was discharged on March 9th at 13,000 platelets. I was re-admitted on March 15th with a platelet count of 5,000 again. This time I was treated with IVig and Solu-Medrol over two days. I was discharged on March 17th with a platelet count of 19,000. On Monday, March 19th, my platelets were 39,000!! Ecstatic!!

On Friday, March 23rd, my platelets were back down to 19,000. My hematologist had me come in for two days of outpatient IVig on March 27th and 29th. On March 30th, my platelet count was only 8,000. On Monday, April 2nd, my platelet count had dropped to 1,000 and I was admitted to the hospital once again.

At this point, we all agreed that I needed a splenectomy as I was not responding to any of the treatments. I had my spleen removed on Friday, April 6th and was given two 5 packs of platelets during my surgery. Post-op my platelet count was 29,000. On Saturday, April 7th, my platelet counts were 17,000, 10,000 and finally all the way down to 5,000. I was also bleeding internally because I wasn't clotting. My surgeon has performed over 300 splenectomies on ITP patients and I was the first patient of his this had happened to.

I was moved to the ICU on the afternoon of Saturday, April 7th. On Sunday, April 8th at 1:30 am I had emegency surgery to remove over 2.5 liters of blood and a small bucket of clots from my abdomen. Prior to the surgery, I was given a shot of Nplate to help boost my platelet production. The surgery took over 4 hours to perform. Had I not had the surgery, I would have died.

I spent 6 days in ICU recovering from the surgery. During this time my platelets never went above 9,000. I spent another 7 days on a regular floor where I received another IVig treatment, another Nplate shot, Vincristine (a chemotherapy) and finally had my first treatment of Rituxan on Wednesday, April 18th. I was discharged on Thursday, April 19th with a platelet count of only 6,000. The doctors told me I could remain in the hospital and wait, possibly months, for my platelet count to increase or I could go home and live my life. After 19 days in the hospital, I chose home.

Since then I have had 2 more doses of Nplate. Each dose higher than the last. My dose today was 750 micrograms. I have also had a second dose of Rituxan and am going in this Thursday, May 3rd for my third dose. My platelet count today was 3,000. I know the Nplate is doing something because last Monday, April 23rd, my platelet count was 3,000 when I received 500 micrograms of Nplate and on Wednesday, April 25th when I came in for Rituxan it was 7,000. I did not get any bloodwork between Wednesday and this morning so I don'tknow what my platelets did but I did notice petechiae and blood blisters all last week and over the weekend. My wife and I were disappointed but not surprised at the platelet count of 3,000 today. We are guessing it will be somewhat higher on Thursday when I go in for the Rituxan but are sure it will drop again.

The doctors have told me that since I have failed every treatment they have given me, my odds of success are diminishing. I don't know what treatments remain and I have not been able to find ANYONE in a situation similar to mine.

I would love to hear from anyone that has gone through something similar to my situation or knows someone who has. I am desperately seeking answers and support. My doctors have two patients that have chronically low platelet counts, but they DO respond to a treatment, their counts go up and then crash. They are treated and their counts go back up again.

My platelets have not been in double digits since March 23rd.

Thank you in advance for any information and/or support you can offer.

Tim:

You are having a tough time. I HAVE seen other people with stubborn ITP that have achieved remissions, but it took time.

First of all, both Rituxan and N-Plate can take some time to work. Most people do not see a response from Rituxan for 4 to 12 weeks after the first infusion. I wouldn't count that out yet. My counts were between 3 and 11 when I had my first 3 infusions, then went to 150 the week of the 4th infusion. They stayed above treatment level for a year.

As far as N-Plate, sometimes it's just a matter of getting to the dose right. You do know that N-Plate is a maintenance drug, right?

Tim,
What Sandi said - Rituxan takes time - 4 weeks is usually the least it takes.

Also I'm wondering, you have very low counts but other than as a result of the surgery are you having any symptoms of bleeding? We have many here who go on with there lives with extremely low counts. There's a bit of worry when you are below 20,000 but we have many examples here of living that way without serious problems.
Erica

Thanks Sandi. I know the Rituxan does take time and am trying to be hopeful. I wasn't aware that Nplate is a maintenance drug. I am very concerned about the long term effects of Nplate on bone marrow, which right now is functioning properly and clean smears, chromosome testing, etc. How often do you take the Nplate?

Erica,

I haven't been above 10 for a month except for a few hours while I was getting infused right after my splenectomy and didn't last. I would be happy to get to 20. What keeps me out of the hospital is I haven't had any bleeding other than very very slight nosebleeds. My doctor is comfortable with my wife and my understanding of what is going on and what to look for. Plus, I have a supply of 1000mg Amicar that helps clotting in order to get me to medical care in case of emergency.

I worry of never getting out of the danger zone of sub-10 and trying to live a life.

I have never used N-Plate. After my last Rituxan (7 years ago), I got a remission and it stuck. I have secondary ITP - Lupus is the primary disorder, so treating Lupus, which was diagnosed 8 years after ITP, probably also treats the ITP.

N-Plate is supposed to be used once a week and the goal is to maintain counts around 50. There is a clotting risk with N-Plate (which you may already know) and when you add in other meds and splenectomy, that risk goes up (even with low counts).

I wouldn't worry about bone marrow for now. All studies so far have shown that any problems reverse when the drug is stopped. Also, it can take a long time for that to happen. Some people have been on it for a few years and have not had any problems.

Tim - here is one story I dug up for you re: a seemingly refractory patient.

www.pdsa.org/forum/5-newly-diagnosed-a-frequently-asked-questions/20292-hospitalized-and-treatment-moving-fast.html

TimSt66 wrote:
I worry of never getting out of the danger zone of sub-10 and trying to live a life.

Tim: two comments, which I hope are helpful. First, remember that 99% of ITP patients eventually find a treatment that works. Maybe you are the 1%, maybe not, but many treatments take time; often, with ITP, things that happen fast are bad -- good things take a while. It sounds like you have good doctors and are getting good treatment. Print out the treatments list from the main PDSA page and talk with your medical team about every one.


Second, I have only ever found one treatment that works for me -- IVIG will get my counts up for about 72 hours. It's not really a "treatment" and we use it only in an emergency. I have lived most of my life with counts around 15. Everyone is different -- some people bleed at low counts, some do not. In the teens, I'm usually ok; single digits I'm not. So I focus on double digits. I don't post much about my counts, because I don't want newly diagnosed ITP'ers to forget that 99% of us find good treatments. The bleeding sucks, I've been there (trust me). But I am breathing proof that it is possible to live a long and fruitful life with counts that instantly would freak out any ER doc.

Like Rituxan, Vincristine is not fast acting. Were I you, I would seriously consider continuing to take those two. You also could consider trying higher dose steroids; I've been as high as 2,000mg a day. Didn't work for me, but, at least for me, it was worth a shot. You could also try different types of steroids. Have you looked at Promacta?

Hey steve! Sure miss you around here. I wish I knew how you were really doing!

Did you mean that Tim should consider Rituxan and Vincristine or Rituxan and N-Plate?

Hiya friend. Great to see you again. I am doing fine. H ad a bit of a bout of low counts here but seems to be back a bit. So no complaints. I try to pop in here as often as I can. I don't post a lot but I read everything!

I hope Tim is doing ok.