ITP Facts

I was watching a DVD of the last ITP conference last night and thought I would share some of the information that I found. I have not seen any of this in the articles, so here it is. I was half asleep though so will watch this Disc again tonight to get better details.

We know that ITP is antibody mediated or T cell mediated. What I didn't know is that there are four identified antibodies and three different types of impaired T cells. A patient could have one of those or combinations of those. They can affect production or destruction. This is the reason why finding the right treatment is so difficult; because different antibodies and impairments respond to different treatments. No one knows which a person has or what combination would work to treat it. That has yet to be determined and the tests for those cannot be done on the ITP population yet, only in research labs.

We often ask why some people bleed at low numbers and others do not. There are two reasons that I know of: 1) many people with ITP have tiny platelet fragments called microparticles. Those occur when antibodies cause platelets to break up into pieces. The fragments are quite useful to aid clotting and cannot be counted. 2) some people with ITP have impaired platelets. Something along the way signals the platelets to stop doing their job and they don't do what they are supposed to do. Those people will have more symptoms.

After I watch it again, I'll be a bit more specific with the info. Will update tomorrow.

These are the Antibodies:

IgG Anti-GPllbllla
IgG Anti-GPllbllla (HIV/HCV ITP)
IgG Anti-GPlblX
IgM Anti-GPllbllla

These are the abnormal T cells:

CD4+ T cells
CD8+ T cells
Y/o^- T cells

Statements from the DVD: Everything in bold is straight from the DVD.

Studies have shown that Prednisone and IVIG do not stop destruction; they aid production.

I did not know this.

There are new classifications for ITP. They no longer use 'chronic' or 'acute'.

The Classifications are:

Newly diagnosed - within 3 months of diagnosis
Persistent ITP - 3 to 12 months duration
Chronic ITP - longer than 12 months
Severe ITP - Presence of bleeding symptoms requiring intervention.

This is actually a good thing. People took the 'chronic' and 'acute' thing too seriously. If they had ITP longer than 6 months, they panicked and assumed that ITP would be with them forever since they moved into the chronic label. Some people even counted the days. All it really meant was that the patient had ITP longer than 6 months. Period. Chronic does not mean for life or forever. It just means 'longer than 12 months'.

ITP is considered when counts are persistently below 100. 150 is not the number anymore.

If a patient has no detectable antibodies, it is assumed that the person has T cell mediated ITP. (My words: However, there could still be undiscovered antibodies. There were only 2 or 3 when I was diagnosed and the T cell theory wasn't even known then).

IVIG does not work for T cell mediated ITP.

Some antibodies are sensitive to a treatment and some are resistant. Obviously, this is why some patients respond or do not respond to a treatment when another patient does. They are trying to figure out which antibodies respond to which treatments. This will give us all a more tailored treatment approach in the future.

N-Plate

Some patients have been on this drug for 10 years. There have been no new adverse effects reported and efficacy persisted. There has been a very low rate of reticulin in the bone marrow.

The first splenectomy performed for ITP was in 1916. Only 8% of patients currently opt for splenectomy.

It was always assumed (and still is by many doctors) that if the bone marrow biopsy shows plenty of megakaryocytes (produces platelets), that patient is producing platelets. It is now known that ITP patients can have a normal amount of megakaryocytes, but antibodies penetrate them and they do not produce a sufficient quantity of platelets. This is why the bone marrow biopsy is not an effective way to diagnose ITP, nor does it prove that a patient is producing enough platelets.

I probably have T cell mediated ITP as I apparently have almost no CD8 T cells at all. IVIG didn't work for me so my experience supports that theory too. I have a brilliant immunologist who has tested everything and has now sent my blood off for genome sequencing.

I'm surprised at the statistic of 8% splenectomies. We seem to see more here.

I was surprised by the 8% too, but that's what he said. It does seem like more than that.

You must have a great doctor, Ann! Let me know the results unless you think it's too personal.

That was info from only 2 discs. I have five more, but probably won't watch any for a few more days. I only do it when my husband is out of town. He could never sit through that.

Great information - can't wait to read what else you derive from your viewing - I may have to get the dvd's myself.

Thank you Sandi!!!

Fascinating info. Please keep all the quotes and content from the conference videos coming.

I will. As fast as I can watch them...

Sandi,
Thanks for all of this incredible information. I think you also posted which treatments seem to work for those with T Cell mediated as opposed to anti body mediated, but I can't figure out where that was posted. Can you repost here?
Many thanks as always.

I'll post it tomorrow.

From Ann:

What did seem to be fact was that there is now accepted to be two types of ITP, antibody mediated and cytotoxic T-cell (killer) mediated. Each has its own mode and its own treatment options. Antibody mediated seems to respond to rituximab, IVIG and cyclophosphamide while T-cell mediated responds to steroids, mycophenolate and ciclosporin.

That's a puzzle to me then - Dougie responded to IVIG but not really to steroids or Rituximab

Sandi wrote: From Ann:

What did seem to be fact was that there is now accepted to be two types of ITP, antibody mediated and cytotoxic T-cell (killer) mediated. Each has its own mode and its own treatment options. Antibody mediated seems to respond to rituximab, IVIG and cyclophosphamide while T-cell mediated responds to steroids, mycophenolate and ciclosporin.

so, new question - can one have both types at one time?

Yes.

Alison, what counts did Dougie reach with IVIG? IVIG really is known to affect antibody elimination in some way so you would think that Rituxan ought to work too, unless somehow the antibody production isn't by the B cells. I don't know enough to know the answer. It would have been a good question to ask the experts.

Ann, based on the conference DVD, I don't think they have the answers to that yet.

Hi Ann, I can't really remember, but off the top of my head, I think his count was about 250 7 days later. His Dr wrote rituximab off as failure. Steroids in very high doses seemed to be partially successful (as in got his count to about 50) but after the first week they didn't work. I am thinking of going to the ITP conference next month because it's quite nearby so maybe I will ask.

From reading the posts, it doesn't seem that very many people are treated with cyclophosphamide, mycophenolate or ciclosporin. Is that true? While everything has side effects, from the limited research I have done, these three seem less likely to cause remission with many unpleasant and common side effects. Am I on the right track?

Yes Ali, you should ask. But I bet they won't know. It will be interesting what they say though.

Emily, mycophenolate is commonly used but you are right about the other two. Cyclophosphamide is very heavy duty and I wouldn't use it myself. Ciclosporin is used by children with ITP but not so much by adults.

I did not respond to either steroids or Retuxin, but do respond very well to Promacta. So which type do I have (or can't you tell from the info you have - I get very confused with all the medical names of the medicines)?