ITP with DVT and xarelto

Hello,

I am new to this forum and have been facing some interesting but confusing diagnoses within the last year. I have had low platelets "documented" for about 17 years, it may be longer but was not found until I had my son. My spleen was enlarged and some of my other numbers were out of whack too. Last December, I had knee surgery and subsequently developed a very large DVT (blood clot) running the full length of my calf up into my middle thigh - it was completely blocked. My platelet count at the time was about 59. They started me on Coumadin and started to keep track of my platelets due to the levels (nothing alarming). It was very difficult to maintain a therapeutic level of Coumadin and the clot was not resolving and at one point had started getting bigger again. So the Dr changed me to Xarelto - which has its own set of issues. The Dr believes I may need to be on this for life due to my complications of the blood clots continuing to form.

My concern at that time and still today is my low platelets in addition to taking blood thinners, my blood continues to clot very quickly. The insurance will not pay for me to have any extensive blood workup because its about 6000 dollars. In the end, I was given the diagnosis of ITP even though I really have non of the symptoms (bruising, petechial).

My last two labs were 52 and 53. My Dr has started me on a short term steroid treatment to see if the platelet count will increase. Does anyone has any suggestions on what I can do or have you heard of similar stories?

Hi Becky. Welcome to the group. Experiencing blood clots with ITP is more common than you'd think. There are several reasons for that. There have been a few people here over the years who have juggled low counts and blood thinners. The good news is that they all manage to handle it successfully, although it is not easy.

There are four common reasons I know of that can cause blood clots in an ITP patient. None of them require extensive testing, just a few fairly common blood tests.

1. Antiphospholipid Syndrome (APS) - Thirty-one patients (37.8%) were found to be positive for Antiphospholipid Antibodies at the time of the ITP diagnosis. Eleven ITP patients in our study experienced thrombosis (6 patients had venous thromboembolism and 5 had arterial thrombosis). We noticed that thrombotic complications mostly developed after the platelet counts had exceeded 100 × 109/L after methylprednisolone therapy or splenectomy in ITP patients with persistent APA positivity. These data are consistent with the suggestion that severe thrombocytopenia might be a protective factor for the development of thrombosis in APS. A few people here have had this diagnosis (I also have had the antibodies).

www.bloodjournal.org/content/98/6/1760?variant=full-text&sso-checked=1

2. Factor V Leiden - (FAK-tur five LIDE-n) is a mutation of one of the clotting factors in the blood called factor V. This mutation can increase your chance of developing abnormal blood clots (thrombophilia), usually in your veins. Most people with factor V Leiden never develop abnormal clots. However, some people with factor V Leiden develop clots that lead to long-term health problems or become life-threatening. A few people here have also had this diagnosis.

www.mayoclinic.org/diseases-conditions/factor-v-leiden/basics/definition/CON-20032637

3. Splenectomy - Within the ITP population, potential mechanisms by which splenectomy could increase the risk of VTE were recently reviewed39 and include loss of the spleen’s filtering activity, allowing for increased circulation and exposure of damaged red cells, cholesterol, and C-reactive protein. Animal models have suggested that splenic macrophages regulate inflammation40 and mobilization of splenic macrophages could promote thrombosis. Candidates for splenectomy are likely to be patients with relapsed or refractory ITP, a cohort also likely to be receiving aggressive medical therapy in the form of higher levels of immunosuppressive drugs and repeated hospitalization. It is possible that these risk factors increased the incidence of thromboembolism.

www.ncbi.nlm.nih.gov/pmc/articles/PMC3674676/

4. Platelet microparticles - Researchers in Spain measured and tested the clotting potential of microparticles (very small bits cells) in the blood of people with ITP and found that the microparticles in these patients were more prone to form blood clots than the microparticles in the control population. This was also true of those ITP patients who had a splenectomy and were in remission. The clotting potential of microparticles may be helpful in preventing bleeding, but this condition may also make people with ITP more prone to heart attacks, strokes, and clots in their veins than might be expected.

#3707 “Increased Microparticle-Linked Procoagulant Activity In Patients with Primary Immune Thrombocytopenia”

There is no available test for this.

Tests for the first two:

1. Physicians use a combination of clinical symptoms (see above) and laboratory tests to diagnose APS. The common blood tests for antiphospholipid antibodies are as follows:

Anticardiolipin antibodies (IgG, IgM, and IgA)
Lupus anticoagulant – a panel of blood clotting tests that may include the dilute Russel Viper venom time (dRVVT), lupus aPTT, mixing studies, and hex phase phospholipid test, platelet neutralization procedure
Antibodies to b2-glycoprotein I (IgG, IgM, IgA)

2. Two types of tests can be done:

Activated protein C resistance test. Your blood sample may be tested to determine whether your blood is resistant to activated protein C, one of the anti-clotting proteins that help control factor V. This is known as an activated protein C (APC) resistance assay. If your blood is resistant to activated protein C, you likely have a mutation in the factor V gene.

Genetic test. A genetic test either is done as a secondary test to confirm the results of the APC resistance test or is done alone to determine whether you have a factor V gene mutation. If you're already taking medication for thrombosis, you're likely to have only the genetic test because blood-thinning (anticoagulant) medications interfere with the activated protein C resistance test. The genetic test also can determine whether you've inherited one or two copies of the gene mutation.

Unfortunately my insurance has denied any further testing. I had one test done by my PCP and he said I had lupus anticoagulant but then the hematologist said you can't be on blood thinners when they test for this so he felt it was inconclusive. I am not sure what all tests they have done - DR does not seem very concerned at this point.

I really haven't experienced any issues with this - just trying to make sense of it. I have not had any counts above 80 in the last 20 labs I've had in the last 18 months. I do not even know if I am experiencing any symptoms - I read somewhere that some feel very tired and that would be the only thing I truly can attest too but do not know if that is even related. I am naïve in this whole area and have done very little research. Maybe if my counts went up to normal levels I could tell if I was missing/feeling something ... lol

Thank you for your reply -

Becky:

Here is some info on testing for the Lupus Anticoagulant while on blood thinners.

patientblog.clotconnect.org/2013/04/21/testing-for-clotting-disorders-can-it-be-done-while-on-blood-thinners/

I cannot believe that your insurance won't authorize those tests! They are pretty important. The Lupus Anticoagulant can be the worse of the three APS antibodies, and it would be helpful to know if you have it or not. It looks like testing while on Xarelto is a bit more reliable than testing while on Coumadin.

I guess as long as you are not having symptoms, can keep your INR within range and not have further clotting, you're doing ok.

Sorry to hear about your insurance woes. I've had some similar experiences, and would suggest the following.

You can insist that your health insurance company provide you with a "peer-to-peer review" regarding the testing your doctor says you need. Your doctor then talks with a doctor who works with the insurance company, and they come to a decision. I've gone through this, and it requires little more than patience. Also, I know a physician who does this kind of work, and she's reassured me that most reasonable requests end up being approved. You just have to push to get the review going.

If you've already tried this and not succeeded, then you can contact your state health insurance board and initiate a review process with them. That's more work and requires more patience, but still can get you what you need.

Good luck, and let us know what happens.

I can't believe that even happens. I've always had all tests approved, and there have been many! It's unreal that people can't get proper diagnoses in this day and age. I've had those same tests quite a few times. All I have to do is ask my doctor to order them and go get the blood drawn.

I am waiting to see what the results of this short term steroid treatment. I am not even sure what he wants to see - if it does raise them, then what?

what are the long term complications if ITP goes untreated? This will be my third doctor in this area -- the first two, were kind of like ... why are you wasting my time. Made me feel very bad - I know there are other patients who are suffering and need way more attention than I ... but an ER doctor about freaked out when he saw my platelet counts and how much Coumadin (at the time) - I was taking but my INR was only 1.3.

Prior to my surgery in December last year my plateles were 58 .. dr was okay with proceeding .. my INR was 0.9 even with the low platelets ... 7 days later I have a huge clot. UGH.

I don't want to waste anyone's time - but I also have to make sure I am educated and wont have the "hind sight is 20/20" in a few years.

Becky:

If you were not on blood thinners, your platelet count would not be a big concern. Most people do fine above 20k. But you are on blood thinners and your count is a concern because it needs to stay above a certain number to avoid bleeding. Sometimes that number is 50k and sometimes it is 75k depending on the blood thinner and the doctor.

I can see an ER doctor being overly concerned....they are not familiar with this type of problem. We see ER doctors freak out all the time about numbers that some consider pretty darn good (even if it is 15k). Don't let that upset you. But on the other hand, the other doctors should not make you feel like you are wasting their time because you have a delicate balance to maintain here. Your counts alone are not cause for concern but with the added blood thinners, it is a problem that needs to be managed.

There are no real long term consequences from not treating ITP as long as bleeding does not occur. At this point, you need to keep your counts high enough to avoid bleeding and keep your INR in a certain range. Those are the goals that need to be met and you do need a doctor who will help you with med dosages to meet those goals.