New article

bloodjournal.hematologylibrary.org/cgi/content/abstract/116/20/4307?ct

On behalf of the medical-linguistically-challenged--ack! Can anyone translate this into standard English?

Does the article treat not making enough platelets as opposed to the condition that most of us have--we make them, but our bodies destroy them?

Karen:

The article says that most people with ITP also do not produce sufficient platelets. The theory that ITP is based only on destruction is long past.

Sandi,

What about those of us who respond fantastically to IVIg? Two or three days after my last infusion my counts shot up to 121. Wouldn't that indicate that I'm producing platelets adequately but not able to maintain them in the bloodstream?

Sandi,

What about those of us who respond fantastically to IVIg? Two or three days after my last infusion my counts shot up to 121. Wouldn't that indicate that I'm producing platelets adequately but not able to maintain them in the bloodstream?

I think what Sandi is saying is it could be distruction it could be production - but then I shouldn't put words in Sandi's mouth.

I'm not going to read the article - sounds like one needs a medical degree to get through it - but wouldn't one know via a bmb if there was a production problem?

Hauna:

I can't begin to explain how or why you'd respond to a treatment and how that corresponds with a persons destruction/production problem. I'm not even sure doctors understand why certain people respond to certain treatments and not others.

All I know is that they are discovering that a majority of ITP patients also have production problems. It could be that some patients have a greater degree of production problems than others. It could be that IVIG also helps those with production problems because of antibodies...I don't know.

If they were able to predict who had either or both problems, they would be able to tailor treatments based on that. I don't think we've gotten quite that far. Good question though!

Also many people gets long time remission after splenectomy. So in that case its only destruction problem.What you say...

That seems to make sense, as when you have a splenectomy your body is still producing antibodies but has lost it's ability to "eat" the platelets, right?

No, not necessarily. If that were true, splenectomies would always work. Sometimes the liver and bone marrow take over destruction.

My son Joshua had a BMB, and the docs said that he is producing platelettes and that its a distruction problem....were they lying, can they really not tell this from the results of the BMB???? Im so confused but thats not hard to do! LOL

No, they weren't lying; they probably just don't know. I actually had Dr. Bussell explain it to me once - why production problems do not show up on the BMB. For the life of me I can't explain it although I do grasp the concept.

It's something like this: Megakaryocytes in the bone marrow produce platelets. During a BMB, megakaryocytes are evaluated and are usually plentiful in number. At that point, it's assumed that because the megakaryocytes are fine, platelet production is also. However, megakaryocytes can be either damaged or prone to antibodies that hinder production, and that's where the problem lies. The problem occurs a step after the BMB evaluation.

www.itpscience.com/5801_bone_marrow.html

www.ncbi.nlm.nih.gov/pubmed/10092918

I'd guess that 99% of the people with ITP on this board report a BMB that states that production is fine. The other 1% are diagnosed with MDS or another medical condition. No one has ever said that their doctor told them production wasn't normal. No one. Production is always fine (see above). Recently published reports state that a large percentage of people with ITP have production problems, yet no one gets that information on their BMB results. If you read the data regarding production, they don't use a BMB to test that. Well, they might obtain samples, but it goes through different testing than the regular BMB tests that most of us have done.

The article that I attached does state that megakaryocytes can be lower in number, so there are various things going on with production problems in those with ITP.

However, in the early 1980s, autologous platelet survival studies from several laboratories showed that in approximately two thirds of ITP patients, platelet turnover is either reduced or normal, not increased, as would be expected if platelet destruction were the only mechanism causing thrombocytpenia. Because megakaryocytes express GPIIb-IIIa and GPIb-IX on their surfaces during maturation and because most ITP autoantibodies react with one or both of these glycoprotein complexes, it follows that autoantibody binding to megakaryocytes could interfere with platelet production and release from the bone marrow either by causing intramedullary megakaryocyte or platelet destruction or by interfering with megakaryocyte maturation.

bloodjournal.hematologylibrary.org/cgi/content/full/103/4/1364

Megakaryocytes are bone marrow cells that produce platelets and other blood cells. In the past, studies have shown that 30% to 50% of patients with ITP have a reduced rate of platelet production. Now scientists in the Netherlands have found evidence that the reduced platelet production may be associated with injured megakaryocytes. They found that the megakaryocytes of ITP patients had extensive abnormalities. These megakaryocytes were surrounded by neutrophils and macrophages (types of white blood cells) indicating an inflammatory response against the megakaryocytes. The megakaryocytes also displayed characteristic signals that cells display when they are dying. The authors concluded that the release of platelets into circulation was depressed in some cases of active ITP possibly due to the action of autoantibodies against the megakaryocytes.

www.pdsa.org/assets/e_news/2004/jan-16.htm


I'm still looking for a better answer for you.

Sandi!

I have to laugh, although with Josh's counts being way down from last week and having a very depressing day, all I have to say to all that typing you just did was
HUHHHHHHHHHHHHHHHH?????????????????? :blink: I would so love to understand all that, but Id be lying if I said I did! Maybe on a day where it isn't snowing, my brain would unthaw and allow the info to sink in, but today, between lower counts and the snow, NO WAY!!!!!!!!!!!!! :laugh: I would love nothing more than to print all that out and proudly present it to hem, but they would beat me with a two by four. When I tell them that I joined this site, they look at me like Im out on a day pass from the psych floor! Our docs do NOT want parents to Google or talk about this on the internet, maybe they are afraid that we'll learn something? Do ya think?
Thanks for trying and Im sure your hands are tired, and Im sorry that Im so slow that I just don't get it....most of it. Hugs to you for trying though!

I'll try again tomorrow if I remember.

I cannot believe that your doctors discourage research! In this day and age, it's almost a necessity.