Jay - I think it's great that you are looking at the big picture here in making decisions. To some extent, I agree with your father in law about long-term bone marrow stimulation. No one truly knows the long-term risks associated with that, although the data from the past 10 to 15 years has shown that any fibrosis will reverse upon stopping the drug and it is generally caught early. The fact that you are able to sustain counts for three weeks between injections lowers your risk since you are not getting the drug every week, and you haven't really been using it all that long.
When my doctor suggested spleen removal (several times), I also looked at the big picture when making that decision. It would have been easy to just get it done and hope that it resolved my ITP, but I kept holding off. My intuition kicked in. At that time, Rituxan had just started to be used for ITP and I didn't have the options of N-Plate or Promacta. Basically I had steroids, Win-Rho, Danazol, and Rituxan. Steroids worked but since they did, I used them over and over until I couldn't do it any more. The side effects were horrible. I didn't respond to Win-Rho and Rituxan worked but I had a bad reaction. Anyway, the splenectomy decision just didn't seem to be the right solution because of long-term risks.
My point is that since you are looking at the future and not necessarily a quick fix for now, I wanted to be sure that you knew the long term risks of splenectomy before making that decision. This is all about benefit vs risk.
1. Splenectomy does not always work and relapses occur over time even if it works initially. If that happens, you're right back to the same treatment choices and they then become riskier. Studies show that splenectomy has a greater success rate in those under 40.
2. Life long risk of sepsis. If the splenectomy fails and you use immunosuppressants for ITP, that risk goes up.
"The major long-term risk of splenectomy is overwhelming post-splenectomy infection (OPSI). Post trauma, the life-time risk of OPSI is 0.1–0.5% and has a mortality of up to 50%.12 The risk of infection is life long as cases of fulminant infection have been reported more than 20 years post splenectomy and is greater with encapsulated organisms -streptococcus pneumonia, nessieria meningitides, and H influenza.29,55 Pneumococcal infection is most common and carries a mortality rate of up to 60%. Infection with H influenza type B, whilst much less common is nonetheless significant, particularly in children. The risk of OPSI is greatest during the first 3- years post splenectomy; greater in children especially under 5 years and who have had splenectomy for trauma (>10% excess risk) than adults (<1%).2,29,56 The risk of infection with encapsulated organisms is increased in immunosuppressed or immunodeficient individuals (e.g. HIV, myeloma, leukaemia)."
www.sciencedirect.com/science/article/pii/S1743919113011175 3. Risk of thrombosis. People with ITP are at an increased risk for thrombosis for various reasons. Splenectomy increases that risk. Using a TPO on top of that raises the risk even more.
"Within the ITP population, potential mechanisms by which splenectomy could increase the risk of VTE were recently reviewed 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 inflammation 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.
"In a prospective analysis of 205 patients with ITP, Aledort et al reported the cumulative incidence of VTE to be 5% in the adult patients. Fifty percent of the VTE cases were in patients who had undergone splenectomy. Bennett et al, using an insurance claims dataset, reported an overall rate of thromboembolism of 6.9% in a cohort of 2783 patients with a median follow-up of 15 months. However, the events included stroke and myocardial infarction, there was no increased risk ratio for VTE, and there was no information on splenectomy. Another prospective analysis of 114 patients with ITP refractory to splenectomy reported an increased rate of VTE compared with the expected rate of VTE in the general population; however, this increase was not significant when patients with other VTE risk factors were removed from the analysis. In contrast, a more recent retrospective analysis of 233 patients with ITP who underwent splenectomy revealed an 8% incidence of VTE in the 10-year period of follow-up, a higher incidence than previously reported and herein.
In a study to evaluate the safety of romiplostim in patients with ITP, 4.9% of treated patients had a thrombotic event. Most patients who had thrombotic events had preexisting risk factors for thrombosis before initiation of therapy. The same group reported a cumulative incidence of 2.4% for the entire study cohort but did not specifically analyze the relationship of VTE to splenectomy."
www.bloodjournal.org/content/121/23/4782?sso-checked=true I thought about the possibility of acquiring another autoimmune disorder in the future or possibly even cancer and wondered how the splenectomy might have an impact on the ability to handle immunosuppressants or chemo. As it turned out, I did become diagnosed with another autoimmune disorder and have been on very strong immunosuppressants for that over the years. It took me 8 years to acquire a lasting ITP remission, but as the events unfolded, I was very glad that I hung on to my spleen. I don't know the long-term risk percentage for a person using N-Plate, but that should be weighed against the long-term risks of splenectomy.
