I've searched the forums and not found what I am looking for, I hope somebody seeing this has something to offer. My hematologist is between waiting another 6 months of weekly/~3-week IVIG treatments in the hope my ITP subsides, or a splenectomy, as nothing other than IVIG has worked to keep my platelets from crashing to below 10. I don't want to do either and am waiting on coordination between radiology and hematology to try a treatment I found in a study, a partial splenic embolization (PSE). Here is a link to the 2018 study:
It was the only scientific study I could find that treated ITP as a primary disease with PSE, all other studies I found use PSE to treat ITP as a secondary disease. I'm trying to avoid a full splenectomy since it is reported to only have between a 60-80% success rate and hope that while the PSE has a 50-75% success rate, it may offer a cure without the risk of a full splenectomy.
Biographical Data: 40 y/o Caucasian male, no other major health issues aside from sudden-onset ITP in June 2019. I also take 200mg daily modafinil for hypersomnia, which I’ve had about 5 years, and treated for ~2 years.
Bottom Line: looking for options/success stories of partial splenic embolization for ITP when all other medications have failed.
Failed Treatments: Rituximab (last treatment August 1st, 2020), Promacta (started 50 mg dose August & 75 mg September, continue to take ), Prednisone (60mg daily for ~8 weeks, tapered off), Platelet Transfusion (successful treatment in ER on initial ITP discovery, Prednisone with Platelet Transfusion and IVIG, had anaphylactic shock on subsequent try with platelet transfusion and since not received as treatment). 15th round of IVIG led to anaphylactic shock and a blood clot. No longer on IVIG treatments (last treatment November 2019).
Successful Treatments: Dexamethasone with IVIG & IVIG alone. Continue with weekly to every 10 day treatments of IVIG. Updated: no longer the case, see above.
History: June – October 2018: In June I had a low CBC during annual military physical (135). Thought to be a fluke, and on return from a deployment re-took (October), found to be low-normal (213). No further medical treatment was pursued by my military doctors, moved on with life.
May – June 2019: In May I noticed hematomas from semi-routine military training activities, and in June 2019 noticed petechiae on my legs/torso. Thought the petechiae were from non-normal trauma I received during training, high g-forces, and planned a visit to the doctors office in a week or so. Mid-June 2019 moved a very heavy object (~300 lbs) and felt extremely unwell, went to the ER, had a platelet count of 2. Received platelet transfusion, prednisone x 40mg, and IVIG 1st dose. Admitted to the hospital for x 3 days, during which x2 more IVIG treatments were given, and long-term prednisone as well (upped to 60mg). Discharged after x3 days, but on the night I was discharged, had an extremely bad migraine, which is not normal for me. Went to Johns Hopkins ER, admitted for another 5 days for the headaches. Note that I often had accompanying spleen pain, for what I believe to be approximately 2 years before ITP symptoms presented.
July – August 2019: Spent several weeks in the hospital, as between treatments I would swing from low-normal (~150s) back to below 10. In July I completed a 4-week treatment of Rituximab while on 60mg daily of prednisone, to no avail. It would take approximately 1 week to 10 days to go from ~200 back down to ~10, when I would be admitted and given IVIG again. Started Promacta 50mg daily.
September – October 2019: Went between 7 and 14 days between IVIG treatments. Took Promacta for a month at 50 mg, then increased to 75 mg with no noticeable effect. Received IVIG x2 weeks in a row, the second week was still at platelet count of 127 and didn’t need IVIG again for almost a month. Not clear if this is b/c of the IVIG while already high count, or if the Promacta affected the elevated platelets.
November 2019: November 18th had my 15th IVIG treatment. Approximately 30-45 minutes later, on the way home developed anaphylactic shock. Spent a few days in the hospital with that, then on November 23rd developed Deep Vein Thrombosis (DVT, aka blood clot) in my left calf. Again spent a few days in the hospital with that. With the IVIG reaction not planning on more IVIG treatments. With the clot am now on blood thinners. I have been on Promacta 75mg daily for the last two months, and since the shock am also now on Prednisone 20mg daily. We are hoping the new combination will keep my numbers up. They have started declining, matching past declines, but we have another week to know if they stay up, at least based on past experiences. We aren’t sure what treatment will be used if they tank again, am I’m not sure how that will work with the blood thinners either. Post IVIG/shock believe the clot was from the IVIG, and had severe exhaustion (~20 hours a day in bed/lounging) which lasted at least 12 days, then like a switch went away on November 30th.
December 2019: was able to correlate IVIG use with dexamethasone or prednisone, use and came to the following conclusions: 7 - 10 days post IVIG my platelets would hit their max, regardless if Promacta was used or not. If high dose steroids were used the max was higher, often in the 400s, otherwise in the low 200s. After the max was reached, I would lose approximately 25 percent of my platelets every 3 days until intervention was required. Unfortunately because of the bad IVIG reaction I had well as the DVT, I had to get a spleenectemy. During the spleenectemy I developed Pulmonary Embolism, and am still recovering from that. Since the spleenectemy, my platelets have been between 1 and 1.3 million count. Still too soon to see how they will level out. My hemotilogist are short of calling me cured, but do think my ITP is in remission, they are tapering me fastest to get off Prednisone to test their theory. Note during the spleenectemy and ensuing 6 or so emergency surgeries I took 3 washed blood units without incident. I have the supporting charts and other information should a member contact me in request.
How My IVIG Symptoms Present:
Below 5: petechiae on legs/torso. Oral purpura in the mornings when I wake (blood blisters), spontaneous bruising on legs and arms
Below 50: “bone” tired, no energy to do anything. Note there is not much literature I can find supporting the fatigue with low platelets. My doctor tells me that while some patients report the fatigue, it is not something they are taught as an ITP symptom.
Below 70: I can “feel” the impact, less energy and tired all the time, like after an IVIG treatment
Post IVIG: that evening and the following 72 hours, no energy, tired. After the first 3 IVIG treatments had severe migraines that had me in the hospital for about 1 week. Now after about 15 treatments the headaches are only minor, but the fatigue remains.
Diagnosed in 2014. I'm a retired renal specialist nurse
Thank you received: 337
There are several more medical options open to you before you consider anything drastic like surgery. Have you looked at the treatments section on this site?
Rituximab can take up to 4 months to work, Eltrombopag sometimes takes a while to get going. Also higher doses than 75mg are now being prescribed see Carcamoc 10's thread.
We cannot change the cards we are dealt, just how we play the hand.
Thank you received: 8
I have read a couple of articles while I was considering that. It's generally safer than complete splenectomy because your spleen is still doing his job. But success rates are lower (For the same reason !)
An experienced surgeon must do that and it's not easy.
If you google it you can find a handful of good articles about that.
The following user(s) said Thank You: globallynomadik
Thanks for the reply. I did see the treatments section, and read about the increased Promacta, 150 mg vs the max dose of 75 mg my hemotologist is prescribing. I'm in between the 3rd and 4th month post Rituximab treatment end date now. I will ask about the increased Promacta option, seeing another hemotologist at Hopkins for a second opinion as well. IVIG wipes me out for about 72 hours after treatment, and I usually go about 10 days between. I'd don't want to continue with only IVIG if possible.
globallynomadik wrote: ... September – October: Went between 7 and 14 days between IVIG treatments. Took Promacta for a month at 50 mg, then increased to 75 mg with no noticeable effect. Received IVIG x2 weeks in a row, the second week was still at platelet count of 127 and didn’t need IVIG again for almost a month. Not clear if this is b/c of the IVIG while already high count, or if the Promacta affected the elevated platelets...
Nomadik, did you continue to take 75mg of Promacta during the month you didn't take IVIG? If so, that would explain the extended IVIG response.
If you can convince your doctor to try higher dose Promacta, that seems like an easy path going forward.
If you haven't seen this thread on spleen embolization, here is a link. I've not seen anyone here with row 4 treatment responses report success with splenectomy.
My rationalization on why splenectomy doesn't work. Obviously, platelets are circulated through the blood and can be destroyed by antibodies in the spleen. But AFAIK, Megakaryocytes are mostly confined to the bone. Thus the existence of the spleen doesn't matter to Megakaryocyte destruction.
Thanks all for your input. I was taking (and still am) the Promacta @ 75mg daily when I had the 3.5 week long high numbers. I will ask about the immunosuppressants mentioned (mycophenolate or azathioprine) as well as the increase in Promacta.
See my post topic Tavalisse has saved my life and given me a new normal life back. I'm similar to you. All else has failed me for 5 years. Almost died from N-Plate giving me an Aortic blood clot because of erratic platlet spike up to over 967. Nearly lost my leg too. Put on Tavalisse a new drug 3rd tier. So for more than a month now no other meds, or steroids, and averaging between 228-340 weekly platelet counts. Still have my spleen. Don't let anybody mess with your spleen until you've at least exhausted everything else. Tavalisse is my lifeboat. I take two pills now a day and have normal folk's 300+ counts! I've been to 0, and in the single digits at least 5 times over the years and in the ER. My mother's side of the family is ridden with autoimmune disease, hence mine beginning at 50 years old. I'm turning 55 in two months. Had chronic ITP for over 4 years. I've had IvIgs at least 5 times, Rituxin, Pomacta, N-Plate for a year or so. Dex steroids as rescue meds when others failed, or had drops outs. Tavalisse is doing great for me. It's unbelievable. It takes at least a month though taking it before you may see the results. It has to build up in your system and begin working in your spleen. I'm on the starter dose of 2x100mg per day. It's $11k a bottle market price, but in august of this year I had three N-plate injections and they were nearly $19k on my insurance receipts that insurance paid the most of so Tavalisse is even cheaper than weekly N-plate. Best of luck! Spread my success story to help others.
The following user(s) said Thank You: globallynomadik
See my topic "Tavalisse has given my life back". Until you get moved to Tavalisse, nothing may work. Nothing worked for me, and I've had ALL short of splenectomy, and N-plate nearly killed me after taking it for a year+. TAVALISSE is my savior! IT's tier 3 so don't take BS from your Dr. or the insurance companies risking you on the other treatments. It's a 2018 drug. Spread the news. I'm as bad almost as they came, and so far I'm saved! Averaging 300+ count every week for a month now. Takes a month or two and then it starts working in your Spleen. YES, it is the first drug to do what a Splenectomy would maybe do. It gets to the root cause and inhibits an SkY protein, or whatever it is that is responsible for marking your platelets for an early death. Spread my story. Look for my post "Tavalisse gave me back my life" in the topic section for more details. Good Luck
Been there done all that and much more. Tier 3 2018 drug called Tavalisse has now saved me! 300+ counts weekly for a month now after graduating from N-Plate, which almost killed me last month with a spike of 967+ in platelets. Got a nice new Aortic stent put in for the mother of all blood clots, and a piece went to my knee and nearly took out my leg, so had to settle for a lower leg fasciotomy! Fun stuff folks! I'm a VETERAN of ITP now! I've been everywhere and all meds, Rituxin 3 times, IvIgs 5 times, steroids, Promacta, N-plate over a year. TAVALISSE is what I was put on after my near death from blood clot on N-Plate. 300+ counts weekly for more than a month now folks! Spread the news. Save others! Yea, I had the blood blisters in the mouth the red dots on the skin, nose bleeding. Yup been there multiple times, ER 5 times with single digits to a record ZERO count once, immeasurable only by hand not machine. THANK GOD for Tavalisse!
Had another dose of IVIG today, still about 12 days between infusions. On the way home started having anaphylactic shock, found the nearest ER and got some epineferin, admitted for the time being. Does anybody know if this reaction will preclude future IVIG treatments? It the ONLY thing that has been working for me, and I never had a reaction from the 20 or so other ones.
For what it is worth, and to hopefully help those in the future, I wanted to add some more recent detail. I had my last IVIG treatement on November 18th. As stated above on the way home I developed anaphylactic shock, then several days later developed a deep vein thrombosis (blood clot) in my left calf, and am also now on blood thinners for that.
The doctors think the clot may be from the IVIG, but seeing as I'm also on Promacta 75mg, aren't able to rule it out. For now I'm on Promacta 75mg and 20mg Predisone, a combination I haven't been on yet, and my numbers haven't tanked. They have started to decline, which mirrors past drops in my numbers, but based on the past have another week before they will get critical if the new drug combo isn't working. We aren't sure what treatment we will use should they go back down, and I'm not sure how blood thinners will work in that whole situation. I have read that my platelets may have been "bigger" with the IVIG treatments, and personally belive the clot had something to do with that, but to be honest am not finding much literature in regards to blood clots, platelete size, IVIG, shock, and blood thinners.
Also, I wanted to add that I have been extremely exhausted, like spending 20 hours a day between bed or lounging on a couch. This exhaustion didn't cease until November 30th, then like a switch went away. Again I'm not sure if this was related to the IVIG/shock or why I had it, but am feeling a lot better now.
Nomadik. Yes, taking either Promacta or Nplate can lead to bigger platelets. Specifically, the Mean Platelet Volume (aka MPV on one's blood test) will go up. And yes, fatigue is a fairly common complaint with ITP.
In past reports here on this forum, those that don't respond to 75mg of Promacta will move on to Nplate. Specifically a higher dose of Nplate. But recently, some folks have moved on to 100 to 150mg of Promacta and will end up responding at that dose level.
As I referenced earlier, Tavalisse is likely a good option too. If you take 75mg of Promacta along with it, your chances of responding should be even better.
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