Diagnosed in Jan 2016, suggestion needed!

I am a 28 years old female college student. I was on a trip to Arkansas at the end of last year. During the trip, I found many red spots on my leg and I thought I was bite by insects. After I came home, I went to see the doctor and he did a CBC. The result turns out to be a shock: pallets count is ZERO! I was sent to the emergency room for pallets transfusion and diagnosed with ITP. Since then I took 60 mg Prednisolone and my response is good. The count go up to 60 and I was sent to home. I continue to take Prednisolone for a few weeks and my count go up to normal: 150-200. So the hematologist advises me to reduce the dosage to 40mg and then 20 mg. Then the pallet count starts to decrease from 180 to 120, 80, 51, then I have to increase the dosage to 40 mg and the pallet count increase to 120 again. Two weeks ago, I found my face has many pimples and I believe that is because of the side effect of steroid, being worried too much on my face, I stop taking Prednisolone by myself for a week without consultation with the hematologist, then my latest pallet counts are 11, I was sent to the hospital for pallets transfusion and the doctor suggests me to take 60 mg steroid again. So what is my next step? Should I change to another medicine such as Rituxan? Should I take high dose steroid (60 mg) for more weeks? I certainly experienced side effects such as unsleeping, fatigue.., but when the dosage is less than 20 mg, I am fine with it.

My mom was diagnosed with ITP more than 30 years ago, she took Prednisolone (I believe) for a few months and recovered, the ITP never came back for her! I hope I could be as lucky as my mom.

Thanks.

Hi Angela! Acne can be a side effect of the steroids, but you should never just stop taking Prednisone! Stopping without tapering can cause adrenal crisis which can be serious, and if severe enough, can be fatal. Please don't do that again.

I think your Hemo is tapering you too much, too fast. It seems like you start at a dose and then get tapered in half. When counts fall, she raises your dose and it starts all over again. You should taper by 5 mg's at a time and platelet counts might hold longer.

If you are tired of the steroids, you could move on to Rituxan. It is a much more heavy duty treatment, but steroids do get old after a while.

I just did a blood test, it seems even i took 60 mg Steroid for 4 days, the platelets count still goes down to 13. So what other options i have in your opinion?

Thanks.

There are a few options, Rituxan being one. Have you discussed this with your Hemo?

Yes, he suggested RUTUXAN, I did a search and find that it is also used to treat cancel and have many serious side effects. i am hesitating now. I thought there is a chance that i could copy my Mom's trajectory, who only use Stenoid and recovered. if i use RUTIXAN, i may get some permanent damages to my immune system.

I read the "ITP and family"( www.pdsa.org/component/k2/item/22.html?Itemid=56 ).

I think what i get may be inherited or familial thrombocytopenia and the treatment may be different. I need to consult with a Heme who is specialized in inherited thrombocytopenia. Not sure i am right or wrong.

Angela, if you are hoping for a remission from steroids alone, your best chance is with the high-dose dexamethasone (HDD) protocol.

www.bloodjournal.org/content/127/3/296.abstract
High-dose dexamethasone vs prednisone for treatment of adult immune thrombocytopenia: a prospective multicenter randomized trial
HD-DXM is a preferred strategy to conventional prednisone as first-line management of newly diagnosed adult primary ITP.

Dexamethasone is a much stronger steroid than prednisone. The 40 mg per day dose is equivalent to at least 250 mg of prednisone, but you only take it for 4 days, then stop. This cycle is repeated up to six times, usually monthly but sometimes two weeks apart. This protocol is very difficult for many people to tolerate, while some find it easier to tolerate for 4 days than taking long-term steroids. One advantage is there is no tapering period involved.

In one study 6 cycles of 4 doses of high dose dexamethasone produced a 90% 15-month relapse free survival rate.
In another study 4 cycles of 4 pulses of high-dose dexamethasone produced an 81% 15-month relapse-free survival rate
(where relapse-free means required no further treatment during the 15-month period).

www.bloodjournal.org/content/109/4/1401.full?sso-checked=true
Therapy with high-dose dexamethasone (HD-DXM) in previously untreated patients affected by idiopathic thrombocytopenic purpura: a GIMEMA experience

I should add that if you think you might have inherited or familial thrombocytopenia, that is a complete game changer. You may have to travel to find a physician who is familiar with that. Sorry, I don't know much about it.

My sister and I have both had ITP. She went into remission after a year with steroids only. It took me 8 years and many treatments. No two cases are the same.

If you want to avoid the toxicity of Rituxan, you could try N-Plate or Promacta. They have few side effects and a high success rate.

Hi Angela,
.
There is a doctor who spoke at one of the conferences about familial thrombocytopenia. It is different than regular ITP. If you look at some of the past conference notes, you might be able to find this doctor. Or you could be just like Sandy who happened to have a family member with ITP. It does take a lot of patience to cope with ITP; just keep educating yourself and you will get there.

I did the Decadron pulses and had first a 4-year remission and then a 6-year remission; but, I still had to have a lot of patience as my platelets didn't go right up. It took almost 4 weeks with very low platelets.

Sandi,
Is the "TPO-receptor agonists" the same as "Promacta"? If i still response to high-dose (60 mg)Stenoid, should i continue to use Stenoid or shift to Promacta?

Thanks.

Angela - yes, the TPO receptor agonists are N-Plate and Promacta. Whether or not you continue to use steroids is up to you. I usually tell people when you are totally fed up with steroids and can't take the side effects any longer, it is time for a new treatment.

After i stopped the Steroid for a week, my PLC dropped to 11 last Wed, i did a plalele transfusion last Wed and started 60 mg Steroid in the same time, here is the PLC number, Wed-11, Thursday-18, Friday-12, Saturday-38, Monday-36. It seems i still response to Steroid. So should i continue the 60 mg Steroid or use RUTUXAN instead?

Can i try High-dose dexamethasone?

Thanks.

I'd try anything that got me off steroids

Angela, I'd give it some thought first. Like I mentioned, I did the Decadron pulses. You take the pulses (high doses) for 4 days, then I think it is waiting 2 weeks taking nothing. Then you do another series of pulses. You do this 3-4 times, depending on your doctor. It did give me a remission.

If you doctor okays it, I'd probably skip the Rituxan and go for the Promacta. It seems to have better results (or NPlate).

What do the others on here have to say?

N plate suits me and seems to be working. Advantages over promacta are no dietary restrictions. Here in the UK we self inject N plate not sure if that facility is available everywhere

That's great! In the US, you cannot self inject--so I think some prefer Promacta (if their insurance covers it). What are your thoughts on the difference between the two? I also didn't know that Promacta had dietary restrictions. What are they?

I think one day in the future, you'll be able to self-inject on the N-Plate.