A type of ITP which induces skin Dermatitis?

Never had blood work in my entire life until I had my appendix out a couple months ago. On hospital admission my count was 12. 'Easy bruising' it is in my case, which I've noticed for at least the past 10 years, many more years likely. Chalked it up to age and taking fish oil tablets.

When I left the hospital, through transfusions, my count was 57. Then the darnedest thing happened. Four days after hospital discharge the count went up to 134. That was music to both me and my hematologist's ears. Then follow up 2 weeks later, the count was back to 12. The same as the hospital admission count.

Now during my hospital stay I noticed that the acne-like non-itching dermatitis around my forehead and scalp I have had since my 20's almost went away. Very surprising. Then it came back sometime after discharge. Through Internet research I found out about the h-Pylori connection to ITP. Also that h-Pylori can cause various skin issues. I wondered. Could the antibiotics they gave me at the hospital cause 1) a temporary remission of the dermatitis, 2) reduce the quantity of h-Pylori bacteria in my gut, and 3) the temporary high platelet count with the absence of the bacteria? I dunno.

My hematologist gave me a 'pulse' of Dex - first line therapy. Beginning count was 18. Wow, that was an experience. It seemed like I instantly became mildly allergic to just about ANY food. Just the act of eating seemed to cause either my eyes to water, my nose to run, or to sneeze outright. All this time I thought my only allergy was to dust. Not with Dex. Even stranger, for about six days around the pulse my forehead dermatitis COMPLETELY WENT AWAY. Then I got my count checked 12 days later. It was 23. Blah.

While I'm having all this fun, I got started on PrevPac h-Pylori eradication therapy. That therapy is now over and my forehead dermatitis has completely gone away, again. This may be a good start, time will tell.

Anyhow, I am looking back now and realizing an additional experience facet. I not only had a non-itching dermatitis on my forehead but I also have an intense itching around my shoulders (right were a seat belt touches your shoulder) that pops up on occasion. To make this long story a little shorter, this itching was EXTREMELY intense RIGHT AFTER I GOT A PLATELET TRANSFUSION last week. That is, I am wondering if the itching of the shoulder dermatitis occurs exactly when my immune system is destroying my platelets. Ack!

Hematologist is now talking about trying IVIG next. Does anyone suppose this is a good next step for someone with a sort of allergy / dermatitis related ITP ?

Have I gone crazy with this disease ?

Platelet transfusions can cause allergic reactions. The reactions can get worse over time. ITP is usually not treated with platelet transfusions. That could be the cause of your rash.

Steroids are great for allergies and is often used for poison ivy, oak, hives, or any other type of rash due to an allergic reaction.

It's hard to figure out what caused what since you had a lot going on at the same time. Were you actually tested for H. Pylori? Was it positive? If not, you may be reading too much into the H. Pylori idea and possible symptoms.

Sandi,
Right and thanks for the pointer about reactions to transfusions. Have not had a bone biopsy yet, so that may be why the nurse was prompting me to get a transfusion with a count of 12. Don't think I want another since I have no bleeding issue. The transfusion experience was instructive though.

On more reflection, I realize that I also often itch around on my back near the tail bone. I have heard that one's long bones are the largest source of blood cells / platelets. So, have to wonder. Am I itching where the long bones (arms and legs) attach to the body because that is where my platelets are entering the body and they are being attacked there? Are these the two places (shoulders and back tail bone) where petechiae often occur? Due to scratching?

Yes, I tested positive for h-Pylori antibody. Have had ulcer flare ups 3 times over the past 20 years but was never treated by a doctor. This long history with ulcers and bruising of mine got me wondering about a possible connection.

To update. Three days after PrevPac treatment end, platelet count went up to 55. Then a few days later my hairline dermatitis started to come back. Then couple days after that, platelet count went down to 12 (normal low for me). As of today, hairline dermatitis is pretty noticeable - again.

My thinking is that the PrevPac treatment failed based on the 55 count followed by the 12. Later this week I'll do the breath test for my gastroenterology to confirm success / failure of the PrevPac. Hopefully it will be positive so the ITP <-> h-Pylori association is firm.

Am I making any sense?

Sandi, quick question. I've read many of your posts It's hard to see what is current. You're taking a lot of Predisone because you have a target of over 100, or over 50, or ?

Or is it more like a switch. Without X dose, one's count drops like a rock ?

I'm taking Prednisone for Lupus, not ITP. My counts have been good for about 10 years now, usually in the 300's. Personally, I'd rather have them much lower.

Update. Since h-Pylori eradication (breath test was negative) my counts have bounced around a bit but have settled, as of today, back down to 14. The earlier 55 count may have been a latent event from HDX treatment. Blah.

FYI I tried a 3.5 day (water) fast last week to try and reset some fraction of my immune system.
news.usc.edu/63669/fasting-triggers-stem-cell-regeneration-of-damaged-old-immune-system/

This one fast didn't really seem beneficial. For me, I am able to get a general direction of counts by monitoring 1) my itch level and 2) my hairline dermatitis level. Using those indicators, I suspect my counts went up on days 3, 4, and part of 5, but then crashed the night of day 5. Blah.

So, now to yield over to my hema doctor. She has been talking of Pred (I think because my throat swelled with Dex) and IVIG. A few things come to mind.

- if IVIG has no lasting effect, shouldn't that be THE LAST drug to try?

If Pred ends up with no lasting affect or is intolerable

- since Promacta and NPlate aren't intended to fix the immune system and are not good long term solutions, should one go directly from Pred to Rituxan? I understand Rituxan has higher risks (eg serum sickness).

Thanks for your feedback...

I wouldn't say that IVIG would be the last drug to try, but it is considered to be a rescue drug. That means that if counts are low and symptoms are getting serious, it could be used to get counts up quickly. It is also used if an individual doesn't respond to anything else. Using it in a non-crucial situation and expecting it to last seems to be a waste of time and expense. It's generally a temporary fix.

I also wouldn't consider N-Plate and Promacta to not be good long term solutions. They can be. Some people have used them for years and have been able to taper off of them successfully with sustained counts. Fixing the immune system doesn't have to be a goal. None of the drugs really fix it; they just change it temporarily. Studies have shown that most people also have production problems so the TPO's can be beneficial.

It's a tough call deciding whether to go with Rituxan or the TPO's. Rituxan can give a remission if a person is responsive to it, but some go through it only to eventually end up on the TPO's any way. The average Rituxan remission is a year, give or take.

Update. Was treated with IVIG for 5 days - monday through friday. That is, the normal dose for 2 days spread out over 5 to lower reaction risks. Didn't have any reaction until the premed dex steroids wore off on Sunday. Then had a fairly bad headache (aseptic meningitis?) for 2 and a half days. Acetaminophen helped only the first day. Was scared to take Excedrin - which was my pre ITP drug of choice.

On the ninth day from IVIG start, count was 308 ! Oh my. Mean platelet volume (MPV) was normal.
That count would suggest platelet destruction stopped at start of treatment and daily production contribution is (308/9=) 34, which sounds good from a megakaryocyte health perspective.

Based on this fun IVIG experiment I can say that my hairline dermatitis is not related to my ITP. The dermatitis is only affected by oral and topical steroids, and somewhat to Prevpac antibiotics. May need to see a dermatologist for this soon.

As for my general itching issue being associated with ITP, as I've described in this thread, the situation is less clear. I'll reserve comment on this until more evidence is in.

Time will tell how long it will be before the IVIG effects fade away...

Dermatitis is usually allergy-related and steroids are used as a treatment. Did you notice that it seemed to go away after taking steroids? It would, of course, probably come back when steroids were stopped.

Allergy related? Ok, thanks. Didn't know that about dermatitis. Interesting. Yes, it comes and goes exactly with taking (dex) steroids, or, applying topical hydrocortisone.

Blah. Guess I need to go back to the drawing board and investigate if certain food, or set of foods, is affecting it. To this point I found nothing except for maybe just sugar. Will try yet another shampoo too...

You should see a dermatologist and have it properly diagnosed. He would tell you how to treat it.

Hey Hal, just checking in for support, just found your thread and will continue to monitor. Best of luck and please update with whatever dietary findings you gleam.

IVIG update. Day 9, count 308. Day 17, count 142.

It appears as though IVIG is mostly a pre Internet age treatment and thus online data is harder to find. Here is a study on repeating IVIG treatments up to 6 times. (LOL, is it coincidental that Dex treatments are up to 6 times as well?)

www.bloodjournal.org/content/82/5/1415?sso-checked=1

Click on "PDF" to see the entire article and not just the abstract.

Take a look at Figure 1. It indicates that if counts fall below 50 before about day 20 then further IVIG treatments are not effective - counts will end up returning to baseline.

Take a look at Figure 2. It indicates that if counts don't fall below 50 until about day 40 that a partial or complete response can be had after up to 6 additional treatments spaced 2 to 3 weeks apart.

Did I miss something in the study? Thoughts?

Hal:

That study was from 1993, when there were only 3 options for ITP: IVIG, Prednisone and splenectomy. I've been here since 1998 and only remember one woman who did IVIG on a regular, on-going basis. She didn't respond to anything else that was available at the time. Everyone else who has tried IVIG usually found that the amount of time it took to keep getting the infusions to keep counts up wasn't worth it. Oh, sometimes pregnant women use IVIG during pregnancy because it is the safest option, but they stop after giving birth. It has never caused a remission that I'm aware of.

I never tried it because I caught on that it was time-consuming and expensive, and the result was not lasting. I was all about getting to work as much as possible. Many of the treatments can be decided upon by lifestyle...if time isn't an issue, a person might choose IVIG as an option until they get tired of sitting in a chair hooked up all the time. It can also become ineffective over time and sometimes the side effects get progressively worse.

What are you hoping for with this treatment?

Sandi, Sandi, Sandi. Don't know what to think here.

Before I even joined PDSA I read this story - which is an IVIG remission story.
www.pdsa.org/join-the-community/personal-stories/item/133-h-pylori-ivig-and-patience.html

Since then I've wanted IVIG treatment. Why, because ITP has many forms/modes and that story is solid evidence that IVIG can have a long term curative effect when h-Pylori is the cause. As I see it, this is 'evidence based medicine'. Also in reading old threads here, when others believed h-Pylori was the cause of their ITP, steroids are mostly refractory for them - which is exactly the case with me. Even more evidence.

So I've got 1) this PDSA story, 2) old published study's about IVIG remissions, 3) testimonies here that IVIG provides neither a partial response or complete remission. What to think? What to think?

All I can come up with is that IVIG's reputation is bad here on PDSA perhaps because of an under/non reporting of its rare positive outcomes. Possible?

LOL, I told my hemo months ago that my goal was a count of 30. I don't think that has changed anything in how she is treating.

Hal - if H Pylori is a cause of ITP, then curing the H Pylori is probably the reason for a sustained rise in counts, not IVIG. IVIG might bump them up temporarily, but I don't think it would be the cause of remission unless the ITP was acute.

There is nothing wrong with using IVIG if that is your choice, it's just not known to cause a sustained rise in counts. It will be interesting to follow your story and see what happens. Keep us updated.

Yes, one would think eliminating h-Pylori would lead to higher counts. But, its vastly more complicated than that. LOL, the subject is ITP here and simplicity is not part of it.

Take a look at this study for how h-Pylori and ITP are theorized to interact. I like this paper because of the nice Figure and caption explanation.
www.bloodjournal.org/content/110/12/3818?sso-checked=true

Notice in the figure there are three theorized generations in how the immune system is working. In the first two generations (acute ITP) what you were thinking is correct - elimination of h-Pylori virus leads to higher counts.

But, in the third generation (chronic ITP) eliminating h-Pylori doesn't help. Due to h-Pylori and it's 'molecular mimicry' to fool the immune system, the immune system antibodies begin targeting platelets directly instead of targeting the virus.

In the PDSA story I mentioned, recall that she had started the last IVIG with a count of 5 and the h-Pylori had already been eliminated. Counts were not going up. That suggests it was third generation effect at work - and thus chronic ITP.

Actually, IVIG was my doctor's suggestion, and I went with the idea quickly. It remains to be seen when my count falls below 50 - which I think she is keying on for further IVIG treatment. Before 40 days or so, probably go on to either Rituxan or Promacta.

Thanks for your interest.

Right, treating H Pylori does not always result in fixing ITP. I've only seen that happen twice here over the years. I know how complicated it all is.

Post IVIG update. Day 23, count 57. Day 32, count 25.

Bahhh humbug. From what I've read, count is falling too fast to consider more IVIG. Doctor said she was looking for more than 30 on the last count. Nevertheless, she offered another round of IVIG or go to Rituxan. So, moving on to Rituxan.

IVIG, just like steroids, are a fun science experiment LOL. Now time to try something whose goal is to fix the disease and not something that just happens to get some into remission along the way. That is, the condition is verifiably chronic.

Now waiting on insurance approval. Expect to start Rituxan next week.

As I recall with Rituxan, some 60% of folks have some kind of response and 40% have a short or long term remission. Further, something like a 4%(healthy) to 7%(sick) chance of a bad outcome. Does all that sound right?

In some sense we roll the dice everyday we get in the car and expose ourselves to life threatening accidents. That percent is small. But deliberately taking a dangerous drug is a bit more profound and obvious as to the risk taking.

A soldier, marching into battle, would be a fool not to be afraid. Likewise, I fear this drug - no doubt. As someone once said, 'all glory is fleeting'. And on that thought: Forward, March.

Good luck with the Rituxin Hal, keep us posted won't you on your journey to higher counts that better bloody stay high!

Hal9000 wrote: Post IVIG update. Day 23, count 57. Day 32, count 25.

Bahhh humbug. From what I've read, count is falling too fast to consider more IVIG.

Hi Hal,

Count of 25 with IVIG after a calendar month I would descibe as spectacularly good. You are definitely responding much better than most. The norm seems to be that counts tailor off within a fortnight or sometimes just a week. Personally I would be ecstatic to have such a sustained response.

Thanks for the feedback rjsmyth. Interesting about IVIG. May revisit that again if Rituxan fails. Speaking of Rituxan, still haven't got insurance approval. Don't know why - haven't called dr office. Perhaps end of year issue or perhaps response to IVIG, LOL, I dunno.

A bit weary of moving on to Promacta/NPlate. I seem to have a background itching issue - if you've had a platelet transfusion reaction, then it is just like that. Have to wonder if the itching would get worse on those drugs since they seek to overload platelet destruction.

The best option is for better drugs, In The Near Future ! How about a Rituxan which specifically targets ITP !

Thanks wquine.

LOL, they had to slap me on the behind, while in my birthday suit, to get me going when it all started. But I've learned a lot since then. It's a struggle, and we are all in it together.

Hal9000 wrote: IVIG, just like steroids, are a fun science experiment LOL. Now time to try something whose goal is to fix the disease and not something that just happens to get some into remission along the way. That is, the condition is verifiably chronic.

Hal - These meds are not fun science experiments unless you enjoy using your body that way. They all have potentially serious side effects that can occur....some are long-term and permanent. You might not know for years if any damage has been done.

Rituxan doesn't fix the immune system. It tricks it into complying sometimes.

Good luck with it. I hope it works for you!

Sandi, no the drugs are not fun science experiments - but that was exactly my point.

I was looking at this document:
www.bloodjournal.org/content/112/4/1147.long?sso-checked=true
which says:
"
Patients, particularly responders, showed restored numbers of Tregs as well as a restored regulatory function upon treatment with rituximab. These results indicate that patients with active ITP have a defective T regulatory cell compartment that can be modulated by a B cell–targeted therapy.
"

Semantics, LOL. Would it be fair to say that my immune system is defective? Can defects be fixed? If the defect is fixed, is the immune system fixed?

Hal9000 wrote:
A bit weary of moving on to Promacta/NPlate. I seem to have a background itching issue - if you've had a platelet transfusion reaction, then it is just like that. Have to wonder if the itching would get worse on those drugs since they seek to overload platelet destruction.

Don't write these off as I would describe you as a responder. The two TPOs are slightly different - Promacta is a bit more akward to take as you cannot eat or drink anything that affects its absorbtion for four hours either side of taking the tablet. Nplate is a lot easier as it is an injection in the stomach (self administered in the UK). Possibility of long term remission is there.

None of the ITP treatments/drugs have no potential side effects. Rituximab can be successful and give long periods of remission, the downside being compromised immune response for up to six months and spectacular infusion issues in a very small number of people.

I have one reservation about Nplate (Promacta did not work for me) in that it was successful for me for three years - side effects were tolerable, but when it seemed to stop working (maybe creating antibodies to it) coming off it may have caused my count to drop to silly low levels causing me to be on IVIG weekly ever since (nine months) and no apparent hope of getting into the safe zone of 50 or above. Recent Bone Marrow Aspiration was negative for any issues.

Chatted with Haematologist last Wednesday and he is going to investigate trying Danazol in the New Year but wants to consult with others first.

My options are very limited at the moment, but on IVIG I don't bleed despite having a count of 1 last Wednesday and 2 a fortnight before that.

Thanks for the info/perspective rjsmyth. In looking through past posts on here, I found someone with what seemed like similar itching issues. Rituxan had put them in a continuing remission. Makes me hopeful.

I've not done any investigating on Danazol. From what I see out of a quick search, it sounds good. Found this.
www.nejm.org/doi/full/10.1056/NEJM198306093082306

Isn't it capable of putting folks into remission? Hard to beat that, heh ? Also, good as long as liver does not get out of wack? Did doc say anything about how it might take to become effective?

Hmmm, maybe something for me to try if Rituxan fails...

Hal9000 wrote:
Isn't it capable of putting folks into remission? Hard to beat that, heh ? Also, good as long as liver does not get out of wack? Did doc say anything about how it might take to become effective?

I think it is one of the less used treatments - for women being a synthetic hormone (testosterone) it can be bad news unless specific conditions are indicated and can result in deepening voice, body hair growth and a complete non starter if pregnancy is a possibility. For younger men (I am 63) it can cause fertility issues, but appears to have had some success with refractory ITP patients.

It can take some considerable time to work (3/4 months) with many patients dropping out with side effects. If I do end up taking it I will keep the community appraised of my progress or lack of!

Have you done a search here on the word Danazol?

What really blows me away rjsmyth is your declining response to IVIG. If you found someone that has been on here and complained about a declining IVIG response, and what drug eventually worked for them, might be a golden piece of information.

Did you ever have a full response to IVIG - like when you first had it?

Hal9000 wrote: Have you done a search here on the word Danazol?


Did you ever have a full response to IVIG - like when you first had it?

Not much info on Danazol probably because it is unfriendly towards the ladies!

I did have a full response to IVIG back in 2011 peaking at around 185 at the end of a week, then tailing back down after another week.

Gone back to weekly infusions (50g) instead of the double dose (100g) fortnightly.

I am writing this on my tablet in the hospital whilst having an infusion.

Update : count 8 so better.