Current evidence supports alternatives to splenectomy

For those who might need some more backup for discussing with their doctor their decision to forego splenectomy: (new article)

www.bloodjournal.org/content/113/11/2386?ijkey=4a51fa2a0a554e36643f499ea1f8de7af514d483&keytype2=tf_ipsecsha&sso-checked=true
Clinical updates in adult immune thrombocytopenia (ITP)
Michele P. Lambert and Terry B. Gernsheimer
Blood 2017 :blood-2017-03-754119; doi: doi.org/10.1182/blood-2017-03-754119
Abstract
Immune Thrombocytopenia (ITP) occurs in 2-4:100,000 adults and results in variable bleeding symptoms and thrombocytopenia. In the last decade changes in our understanding of the pathophysiology of the disorder have led to publication of new guidelines for the diagnosis and management of ITP, and standards for terminology. Current evidence supports alternatives to splenectomy for second line management of patients with persistently low platelet counts and bleeding. Long-term follow up data suggest both efficacy and safety, in particular for the thrombopoietin receptor agonists (TPO-RA) and the occurrence of late remissions. Follow up of patients who have undergone splenectomy for ITP reveals significant potential risks that should be discussed with patients and may influence clinician and patient choice of second line therapy. Novel therapeutics are in development to address ongoing treatment gaps.

Rob thank you for all the great information you post for us. My hemo keeps bringing up splenectomy although I have only tried Prednision and Rituxin as treatments. I just started Promacta so we will see how that goes. I am reluctant to give up my spleen, we are very close.

Good article, Rob. I want to read the whole thing later.

It is your spleen and your decision, not your hemo's! There are many options to try after Promacta -- options that often work when others don't -- and you do have to consider their risks versus the long-term risks of splenectomy. My amateur opinion is to avoid splenectomy if at all possible, and many experts would agree.

I see so much research being done regarding ITP specifically, and autoimmune diseases in general -- much of it too complicated for me to understand or to post -- that I am very hopeful of continued breakthroughs in diagnostics and treatment. New drugs are on the horizon, and older drugs are being re-purposed, especially in combination treatments. I am especially optimistic -- now that different types of ITP are being identified -- that there is a possibility of tests being developed to differentiate between different types of ITP, so that the correct treatment or treatments might be selected without the unnecessary risk of so much trial-and-error that currently is required.

Good luck on the Promacta. It usually works, but you will have the challenge of balancing the dose to minimize the chance of thrombosis, especially if you are weaning off prednisone. I suggest that you keep an accurate log of changes in dosages and counts (including dates), and keep us posted on your progress.

Novel therapeutics are in development to address ongoing treatment gaps.

Yea, I bet there are. That's just a bit of a tease...

I don't think it's a tease, I just don't see it happening in the near future. The immune system itself is barely understood let alone trying to control it.

I have been charting my meds, counts, and hospital stays since my diagnosis in October.
Right now I am only on Promacta, I have been off all steroids for over a month.

Sandi, any idea what a 'treatment gap' might be reference to? The only thing that comes to my mind would be something to do with Tamiflu and those that don't respond to first line treatments.

Think about the study/article this way. The author(s) openly renounce splenectomy, and, they espouse going to TPO-RAs for second line treatment. Also realize that the authors are doctors and can easily be privy to experimental projects that can have lots of promise that few know about. If some 'novel therapeutics' does come out, this article is going to sound like pretty sage advice in hindsight.

Hal, What are you referencing when you mentioned Tamiflu? Has there been a study that shows it helps?
Last hospital stay was due to the flu so I was given IVIG (2 doses) since platelets were down to 3 and Tamiflu. My counts sky rocketed I peaked at 145. Last week I was down to 10 again and giving IVIG (1 dose), I only got up to 17.

DmMann,

Here you can see PDSA search results for Tamiflu related to treating ITP:
pdsa.org/discussion-group/search.html?query=tamiflu&searchdate=all&childforums=1
It is a very intriguing possibility that for some forms of ITP, Tamiflu may be effective.
Most of the evidence is anecdotal, but there is a theoretical basis for it, having to do with desialylation.

That's very interesting D Mann. Take a look at this thread .

From what I've read looks like Tamiflu may help most ITPers at least a little. Who it helps the most are those that have 'GPIb-IX' type of ITP. These folks normally don't respond well to either steroids or IVIG. The reason why Tamiflu seems to help those folks is because it reduces/blocks platelet destruction in the liver.

Both you and I seem to respond well to IVIG and have a weak response to steroids. At this point it is academic, but I suspect that the type of ITP we have is where one has antibodies to Thrombopoietin. And with that, I expect we both will have good responses to Promacta - counts ramp smoothly up in two weeks. The proof is in the pudding though and time will bear the facts. It's just a theory....

Hal:
I would imagine that a treatment gap would indicate patients who have little or no response to any current treatments.