Splenectomy failure; what next?

There's a lot of experience on this site and I'd like to get some feedback from those more experienced in dealing with ITP.

My summary: Diagnosed with Non-Hodgkins B-cell, indolent lymphoma 3 years ago. Developed ITP 11 months ago, began steroid therapy and then had Rituxan infusions in Dec. 2014. Off steroids, platelets would collapse to <12,000. It's not clear if the ITP is secondary to the lymphoma, or if both are being driven by an underlying autoimmune disorder. Age is 56, male.

Had a splenectomy in March and platelet counts initially climbed dramatically. But in a followup last week (April 16), my count had fallen to 2,000 and I was admitted for emergency treatment.

Question now is where do I go from here?

For the short-term, IVIG and steroids are keeping the count up. But the long-term mortality of those with chronic ITP after splenectomy failure is high; the side effects of many ITP treatments are difficult and those treatments often become less responsive over time; and most lymphoma treatments will worsen the ITP should the lymphoma become more aggressive (which may be happening based on a chest scan from last week.)

I've been seen by good doctors at both Emory in Atlanta and MSKCC in NY, but it seems I'm in a box with only bad options.

Anyone else have a similar situation and if so, what treatment routes have you pursued or declined?

Thanks.

Mike

Mike:

I'm sorry to hear about all of your problems. You've had a lot to deal with.

I think the big question is whether or not you have primary ITP or secondary ITP. If it's secondary, you may not respond to typical treatments for ITP. Rituxan has been used for both ITP and certain types of lymphoma, so that can work for both, but you've already tried that. The other treatments such as immunosuppressants (CellCept, Imuran) probably are not a good idea due to being asplenic and being immunosuppressed by chemo, but that is up to your doctor.

You might want to ask your doctor about the TPO's; N-Plate or Promacta. I don't know how often they are used when one has or has had lymphoma, but I did find one article about it.

www.ncbi.nlm.nih.gov/pubmed/20628840

Here is the info re: N-Plate:

Nplate® is a man-made protein medicine used to treat low blood platelet counts in adults with chronic immune thrombocytopenia (ITP), when certain other medicines, or surgery to remove your spleen, have not worked well enough.

Nplate® is not for use in people with a precancerous condition called myelodysplastic syndrome (MDS) or low platelet count caused by any condition other than chronic (lasting a long time) immune thrombocytopenia (ITP). Nplate® is only used if your low platelet count and medical condition increase your risk of bleeding. Nplate® is used to try to keep your platelet count about 50,000 per microliter in order to lower the risk for bleeding. Nplate® is not used to make your platelet count normal. It is not known if Nplate® works or if it is safe in people under the age of 18.

www.nplate.com/patient/how-nplate-may-help/results-with-nplate.html?WT.srch=1

Your situation is very different from the rest here. We don't often have people like you here and when we do, we don't know enough about the situation to be of much help.

Hi Mike,
I hope you become well.
Nowadays, we are in similar position with you. How can you find your treatment way, please inform me. It will be very helpful for us. Because my brother has ITP for 4 months and also he was hodgkin lenfoma 2 years ago.
Thank you.

I'm now on weekly Nplate injections of the maximum dose. I also found that iron supplements helped. Platelets vary between 50-70 most weeks.
Mike

Hi Mike
Our cases sound similar. I was diagnosed with ITP 3 1/2 years ago and 2 1/2 years ago Non Hodgkin lymphoma. My oncologist feels the ITP is caused by the lymphoma. Had a second opinion done at Cleveland Clinic. The ITP specialist agreed. I've done the prednisone, Nplate, Promacta, Rituxin, IVIGs all to no avail. I had a appendectomy this April. My platelets went up to 800 K then over a million. (My family Dr said she never heard of platelets that high.) That's just as scary as tanking. Then in a week's time back down to 6 k. I've mostly stayed under now30 with a couple 100's randomly thrown in. It makes no sense. My oncologist feels surgery was a fail. Surgeon said 4-6 months for stability. I may never have normal readings but may reach a more level reading like 50. That would be wonderful. Last 3 weeks I've been down to 2, 3, 12. I'm frustrated too. I'm doing 2 IVIGs a week. I'm symptom free as far as the lymphoma goes. How did your surgery go? I so didn't want to do it but with the odds being 2thirds successful, it was too good not to try.

Sue - I have seen people here have counts that go up in time from a splenectomy 'failure'. It doesn't happen often, but don't give up hope yet.

Counts near a million do happen - I've seen it quite a few times here. You're right, it is scary. It's odd that your counts dropped so fast after that. Counts that high after splenectomy generally indicate success. I've never seen this happen.

Has you physician done any imag (CT, MRI, etc) to make sure your previous splenectomy actually got it all? Found out just recently that there was a splenule left behind after mine, 3 years ago-it has caused my ITP to rear its ugly head again.