ITP for 3 years and looking for advice on next steps

I am trying to understand how someone is diagnosed with ITP. Since it seems to be a disease of exclusion, I don’t know at what point you say it’s ITP and not something else causing the low platelets. I am a 37 year old female from south Florida. I was officially diagnosed with ITP in 2016. (Although I have had CBCs with low platelets before this date) My platelets range from 20k-60k. When initially diagnosed I was put on prednisone which increased my platelets but once I was taken off they dropped to my typical range. This is the only treatment I have received so far.

I have had a bunch of blood work. I have seen two hematologists and a rheumatologist. Nothing is showing up in any of this testing as to why my platelets are running low. Both hematologists say it is itp. Their recommendation has been to just monitor. If they drop and I start getting very tired (which usually happens when in 20k) then the recommendation from one was Rituximab. The other said possibly a splenectomy. I’m not interested in the latter but would consider Rituximab. However, I don’t want any treatment until I’m comfortable that this is ITP.

I’m concerned that they are saying it’s ITP only because we haven’t done sufficient testing to rule out other things like MDS or some other lymphoma. When discussing my concerns with my hematologist he said the only other test I could do is a PET scan to see if I have a low grade lymphoma. Neither hematologist said a bone marrow biopsy was necessary due to my age and all other labs were in normal range.

I’m not sure if this is the right step or if I should be pushing for a bone marrow biopsy first. Are there any other tests that should be done before settling in on an ITP diagnosis? What was your experience?

First of all you need to know exactly what you were tested for when the blood work was down. When I was was first diagnosed 5 years ago I was tested for an absolute multiplicity of things. Far too long a list for here. All came back negative. I was also tested for HPylori (negative). Bone marrow biopsy isn't as common these days. Over here in the UK treatment with a count above 30 would be unlikely. I have my spleen and it is staying put. I have never had rituximab.
I am doing ok on Promacta.

When I was diagnosed, I asked about a bone marrow biopsy and was told I didn't need it to determine if I had ITP or not. When I went back to my GP after my first blood test, he told me it was one of two things: lymphoma or ITP. I had no idea what that was, had never heard of a platelet before.
I trusted my GP, and since it wasn't something he knew a lot about, he sent me to a hematologist and I trusted him too.
I did Rituxan in 2011 and it put me in remission. One thing to remember, just because it worked for me doesn't mean it will work for you. A lot of people on here have tried it and not had any results.
Good luck with your decisions.

Thank you, I pretty confident we have exhausted all blood test. I have also had the h. Pylori test which was negative. I am trying to understand what other tests are out there other than labs. Or are labs all that are done?

Thanks Cindy. If you don’t mind me asking, what do your platelets run? Did you do a PET scan or anything other than labs before the Rituxan?

Thank you for taking the time to respond. I appreciate it!

I was diagnosed back in the dark ages, 1989 - bone marrow biopsy done and lots of blood work. Options then: splenectomy or prednisone - I decided on prednisone because we were moving overseas shortly after diagnosis, plus no one would guarantee that a splenectomy would be the cure. Still have my spleen.

Know my insurance would never have covered a PET scan for ITP. Wouldn't you think after 3 years cancer would have reared it's ugly head? Some have ITP due to lupus, are you still seeing the rheumatologist? I don't know of any other tests but labs, maybe someone else does.

Good luck!

Kadiagle- It really sounds like your hematologists are knowledgeable. You asked for advice- try to trust your doctors, bone marrow biopsy is not necessary. ITP is a diagnosis of exclusion and can be diagnosed with a CBC. If platelets are the only thing off on the CBC then that tells the doctors something(ITP). If you have red blood cells and white cells low then that points more to cancer. If you have symptoms of lymphoma- night sweats, weight loss, enlarged lymph nodes, fever, rash, fatigue- then they would biopsy or whatever. I don't know all about it but I've had ITP for 10+ years and I'll tell you that over-treating and over-testing can lead to trouble.

Make sure you really know what you are doing before pushing for a PET scan. You might likely find nothing while exposing yourself to a great lot of radiation. I wouldn't want my ovaries exposed if there was any chance of ever getting pregnant. Radiation exposure builds up over your lifetime, so be aware.
www.cancer.org/treatment/understanding-your-diagnosis/tests/understanding-radiation-risk-from-imaging-tests.html
"A PET/CT exposes you to about 25 mSv of radiation. This is equal to about 8 years of average background radiation exposure."

If a person does get the scans and bone marrow biopsy, and they find they DO have lymphoma underlying the ITP and do not have symptoms, then it is called "indolent lymphoma". People can have small amounts of lymphoma that never manifests into anything. Maybe its driving the ITP and maybe the two are not related. So then the question is: Do you treat the indolent lymphoma? Or just watch and worry? The treatment is Rituxin so there you are back to where you started. Or they may opt for chemotherapy which is its own huge set of problems that can do permanent damage.

The reason I know this is that my hematologist told me the story of another patient with ITP who insisted on doing all of the scans- PET/CT/MRI and bone marrow biopsy. They found a tiny bit of lymphoma. She had no symptoms so I believe it was considered indolent. They did Rituxin and the Rituxin did not work to bring her platelets up. My hematologist said there was really no good way to tell if the Rituxin affected the lymphoma. So what did she gain? I guess she knows now that she might/might not have a bit of lymphoma so can watch for symptoms. But knowing things like that can affect how you live your life. I don't know whats right, its a personal decision. Its just very important to be educated in these decisions

Take care- thanks for sharing your story! Everyone has their own path, so if you do decided to do more tests, let us know how it goes. Its all very interesting and helpful.

Kadiagle, I haven't had a test in a while, but last time I got results, I was around the 300k mark. No other test other than the blood work. I had IVIG weekly the first year I was diagnosed on prednisone for several years (at different doses) and a splenectomy. That worked for about a month, then I was back in low numbers. I had heard about the Rituxan on here and mentioned it to my hemo doc. He used it for his cancer patients and was trying to get approval for his ITP patients. There were 3 of us that got it and I'm the only one it worked for.

Poseymint- Thank you so much for your thoughtful advice. You hit what I’ve been struggling with right on the head. I agree, I need to trust my doctors more. I have been struggling with the idea of if I do the PET scan and there is indolent lymphoma, would I want to know. On the one hand, my thinking is the knowledge of it could help intervene before it progressed farther along. On the other hand, the knowledge of it may not help at all and could negatively impact the quality of my mental state. I would probably hold off on medications as long as I could, which means watchful waiting (aka worrying). As you mentioned, I’m also concerned with the radiation exposure. I will probably hold off on a further testing until or unless I start to feel more comfortable with the PET scan or some other symptom appears.

Thank you again for taking the time to help me. I will update here if I move further with testing.

Kadaigle wrote:
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I’m not sure if this is the right step or if I should be pushing for a bone marrow biopsy first. Are there any other tests that should be done before settling in on an ITP diagnosis? What was your experience?

From other discussions here, a good IVIG response is a positive diagnostic for ITP diagnoses. This makes sense since ITP is an immune system disease and IVIG only affects the immune system - specifically gamma globulins.

Also. You may want to look at this video at 16:20 through 40:00 or so. I think this doctor seems to be indicating a good IVIG treatment response, or, a good steroid treatment response, either one, are both diagnostic to ITP.
www.facebook.com/plateletdisorder/videos/1298013283700025/

Hal9000- thank you for your response and thank you so much for sharing the link from the PDSA conference. It was very interesting to hear and learn more about the disease.

Just one thing about lymphoma. Most who have an indolent lymphoma do not have low platelets. In fact they usually have completely normal blood. I have had lymphoma for a few years so I know a bit about it. I can't imagine any good doctor prescribing a scan when there are no symptoms. It really is not a good idea.