Anyone have good info on Splenectomy?

Hi everyone!

I have just finished recieveing my third Rituxan infusion, and my counts are at 8. Unfortunately, my hemo says it doesn't seem to be working, but we'll wait until a week after the 4th infusion to make the final decision. She said the next step is to gett a splenectomy. I asked her about NPlate and Promacta, but she doesn't like to use them in young people because of the risk of liver failure and bone marrow disorders. I'm only 22.

I just feel like splenectomy is such a drastic decision at this point in my journey. I was only diagnosed 3 months ago! Anyone have any good resources on info for splenectomies so I can make a more educated decision? Thank you so much for the help!!

It's very soon to have a response to Rituxan. My counts went something like this during my infusions. 1st - 8k, 2nd - 11k, 3rd - 3k, 4th - 150k. Most people don't respond until later than that and it can take up to 12 weeks to see a response.

I do have some good info re: splenectomies, but it's late right now so I'll post some articles tomorrow, ok?

Considering a splenectomy after such a short time is unbelievable. Even more so in one so young, removing healthy organ that is doing its job. . There are so many treatments available I suggest you stand your ground and keep your spleen. Over here in the UK it wouldn't even be considered this early.
I would ask your haemo for her evidence base and if she follows ASH guidelines
www.bloodjournal.org/content/117/16/4190?sso-checked=true#sec-19

I may have mentioned this before. With ever advancing medicine, NPlate / Promacta shouldn't be considered a forever drug.

IMHO, mrsb04 is absolutely correct. I've not seen a study which indicates the problems with NPlate / Promacta your doc mentions. But I have read here on PDSA those problems were once roomered to be the problems when the drugs first came out and no one knew. They were just guesses then.

Here's a start:

www.onclive.com/insights-archive/immune-thrombocytopenia/risk-of-thrombosis-in-patients-with-itp

fhs.mcmaster.ca/medicine/hematology/ITP-2010/player.html

www.ncbi.nlm.nih.gov/pubmed/23637127

www.ncbi.nlm.nih.gov/pubmed/24942752

Introduction:

Splenectomy was historically regarded as the gold standard for treatment in chronic adult immune thrombocytopenic purpura (ITP). However, the recent emergence of new drugs has deeply modified ITP management and splenectomy is no longer viewed as an unavoidable step in adult chronic ITP in many countries. The estimation of the risk over benefit of this potential curative treatment remains challenging both for patients and physicians. A retrospective Italian study focused on long-term outcome of patients splenectomized for ITP gave reassuring data concerning safety. A recent study from a large cohort of American veterans showed an increased risk of death due to septicemia, pulmonary embolism, coronary artery disease and cancer more than 10 years after splenectomy. We reported here the results of the first single center case-control study evaluating the long-term incidence of splenectomy complications with a minimum follow-up of 10 years.

Methods:

We retrospectively selected in a clinical computer database all primary ITP patients splenectomized more than 10 years ago in our unit. We matched 1 by 1 to non-splenectomized ITP patients based on date and age at ITP diagnosis and sex criteria. Clinical data were then completed from medical charts. All patients were interviewed by phone and a standardized questionnaire was used. Medical records from general practitioner or from Medical care center have been systematically obtained if necessary, especially for deceased patients. Comparison between groups were made using Fisher’s test for qualitative variables, Kaplan-Meier method to estimate incidence and Rank test for comparison of cumulative incidence, with p<0.05 defining significance.

Results:

Seventy splenectomized ITP patients were included (19men/51women) with a median age at ITP diagnosis of 37 years (range: 3-92). Sixty one (87%) initially responded to splenectomy but only 34(48.5%) maintained a sustained response after a median follow-up of 189 months (range:120-528). Matched non-splenectomized ITP patients had a median age at diagnosis of 40 years (range: 3-93) and a median follow-up since ITP diagnosis of 197 months (range: 96-504).Cumulative incidence of thromboembolic events was higher in the splenectomized group (p=0.029) (Figure1). Four (6%) episodes of post-operative portal vein thrombosis were observed, 3 were complicated by portal cavernoma requiring long-term anticoagulation. They tended to present with more thromboembolic events on a long-term (n=7) than non-splenectomized ITP patients (n=3, p=0.113). Two splenectomized (2.8%) and 1 non-splenectomized (1.4%) patients were diagnosed with post-embolic pulmonary arterial hypertension. The incidence of cardiovascular events was significantly higher in splenectomized group (9(13%) versus 2(2.8%), p=0.005) (Figure 2) with 6 transient and/or ischemic strokes in splenectomized patients (none in non-splenectomized).Infectious events were similar in the two groups (splenectomized: 12 (17%) vs 10 (14%)) but infections were more frequent and severe in splenectomized patients. Indeed, 12 splenectomized patients presented 20 infectious events requiring hospitalization, 13 of them were pneumonia (Streptococcus Pneumoniae: n=4, Haemophilus Influenzae: n=1, undocumented: n=9). Five complicated septic-shocks leading to 3 deaths. In non-splenectomized group, 10 patients had 10 infectious events (Pneumonia n=4, Streptococcus Pneumoniae n=1), 7 were hospitalized, none had septic-hock. Incidence of cancer was similar in the 2 groups (splenectomized: 11 (16%), non-splenectomized: 10 (14%).Finally, the mortality rate was not different between two groups (splenectomized: n=14 (20%), non-splenectomized n=9, 13%). Ten (38%) of the 36 non-responders patients deceased, 7 from hemorrhage and/or septic shock. Other splenectomized and non-splenectomized patients died from malignant cancer/hemopathy (n=5), coronary artery disease (n=2),other (n=6).

Conclusion:

Based on this case control single center study, we observed that long-term splenectomized patients have not only an increase risk of life-threatening infections, but also an increased risk of thromboembolic, and cardiovascular events. A long-term follow-up is therefore recommended in this patient population regardless the status of ITP in order to better prevent and manage such complications.

ash.confex.com/ash/2014/webprogram/Paper68906.html

www.bloodjournal.org/content/125/10/1681.full?sso-checked=true

www.ncbi.nlm.nih.gov/pubmed/26547507

Since the development of guidelines for the diagnosis and management of ITP, emerging data on the use of second-line medical therapies to manage patients requiring pharmacologic intervention have resulted in a decrease in rates of splenectomy. Clinical practice is still lagging behind guidelines in following diagnostic evaluations, but has moved beyond those published guidelines in the use of second-line therapies to minimize risks and side effects while providing treatment options with reasonable chances of success. New guidelines will need to address this emerging body of information, and future clinical trials will examine alternative therapies now in development.
www.bloodjournal.org/content/129/21/2829

At the end of two years in retrospective group, almost one-third of 22 patients who underwent splenectomy had either primary failure or relapse. Similarly in prospective group, though small in number, at the end of 23 months itself, three of six patients who underwent splenectomy had either no response or had relapsed. Thus this study questions the utility of splenectomy as second-line treatment after steroid failure. Rather, patient may be given a choice to opt for other form of drug treatment after steroid failure and advised splenectomy as and when unavoidable. Nevertheless, any patient opting for splenectomy should be cautioned that there is a fair chance of relapse later in life. Furthermore, it appears incidence of relapse following splenectomy is higher in Indian patients as compared to that in the Western patients.
www.bloodjournal.org/content/118/21/4690

Depending on the clinical setting, splenectomy is deferred in most patients for at least 6 months. This may be due to patient preference or other active comorbidities and to the understanding that spontaneous improvement or late remission may occur 6 to 12 months after diagnosis; indeed, some patients may spontaneously remit even years after diagnosis.
www.bloodjournal.org/content/115/2/168.full?sso-checked=true

Liver failure?? That is outrageous and not true at all. I have used Promacta and Nplate- and also followed the reports on them since they were in clinical trials 2009. As drugs go they seem to be very well tolerated. Good for you that you are researching! You seem very bright, we all have to advocate for ourselves, do our own research, make our own decisions here. There is such a tendency in doctors to over-treat ITP. When I was first diagnosed I read on the Mayo Clinic website that the ITP patient must be careful because "the treatments can be worse than the disorder." I have never forgotten that and have gone back to it many times, asking myself "which has the most risk, the low platelets or the treatment?"

I don't think anyone, esp someone young, should get a splenectomy during the first year of ITP. You could have a spontaneous remission if say perhaps it was caused by a viral illness. I just don't think a person can know if its chronic for at least a year.

Thank you so much everyone! Getting a splenectomy so soon just doesn't feel right to me, and the evidence you all are providing is just reinforcing my gut reaction.

My counts at my last rituxan infusion were 8. I asked my hemo about NPlate, and she told me that we can do it for 6 weeks to see if the Rituxan starts to work. Once she starts to taper the dosage, if my counts remain above 30, then she says that's fine. Once they drop below 30, then splenectomy is the next option. I asked her what about just not doing anything at all, because I have very few "wet" symptoms, even with my low counts. I've never had a nose bleed during all of this, my gums have only bleed 3 times, and I have very few petichiae and bruises at the current moment. She told me that it's too dangerous to walk around with counts that low, so not doing anything isn't an option.

Ultimately, I think I'm just going to have to stand my ground on this and go for a second opinion. Thanks everyone!!

Oh my goodness - get that second opinion! You don't do N-Plate for six weeks and then hope that counts stay above 30k and then do a splenectomy. It's a maintenance drug! Sorry, I'm frustrated. That doctor seems to be unfamiliar with treatment protocol and side effects.

Time to get a new haematologist methinks.