Article re: Splenectomy and Treatments

bloodjournal.hematologylibrary.org/content/120/5/960.full.pdf+html

Thank you, Sandi, for interesting article. Question: In discussion of unpredictability of splenectomy results, the writer doesn't mention those tests in England which apparently do indicate if source of platelet destruction is the spleen. How useful are those tests anyway? I assume such a test could show the destruction site was the spleen, and then, after the spleen is removed, the liver might take over and destroy the platelets. (My doc assumed that was what happened in the case of my failed splenectomy, though, of course, I never had a test such as the Brits do.)

Funny you should ask. If you read the thread by Rachaely, you will see that she just had a splenectomy that doesn't seem to be successful (although anything can happen). Counts are 37 a few weeks later. She had the Indium and it did show the spleen as the site of destruction.

I've always said the Indium cannot predict success, but it can predict failure (if it determines destruction is in the liver). It's a matter of semantics, but word phrasing is important. Of course, all of this only applies to what happens within a few months of the surgery. It can always fail months or years down the line and nothing can predict that. Personally, I don't consider the Indium very useful for predicting success. I do consider it useful for saving spleens in those who show destruction in the liver. This was the case with Ann, and she chose not to have the surgery.

This is a good paragraph:


What is ITP?

ITP is an autoimmune disease characterized by isolated thrombocytopenia (platelet count 100 000/L) resulting from accelerated clearance and destruction of antibody-coated platelets by tissue macrophages, predominantly in the spleen. Antiplatelet antibodies also target antigens on megakaryocytes and proplatelets, variably suppressing platelet production. Plasma TPO is generally normal or only minimally elevated, primarily because of accelerated clearance via megakaryocytes and platelets. Activated cytotoxic CD8T cells may contribute to thrombocytopenia in certain patients. A potential underlying etiology giving rise to secondary ITP, such as infection with hepatitis C, HIV, or Helicobacter pylori, and coexistence of systemic lupus erythematosus, antiphospholipid syndrome, or common variable immunodeficiency (hypogammaglobulinemia, CVID), is identified in approximately 20% of patients with immune thrombocytopenia. Discovering an underlying cause is important because it may impact the efficacy and safety of splenectomy and other approaches (as discussed in the following sections). Moreover, patients, such as in the case example, are often not thoroughly evaluated for secondary ITP, and an underlying etiology may not be apparent on presentation.


***Please be aware that an underlying disorder sometimes cannot be diagnosed because it is not yet apparent with symptoms and blood work. Some underlying disorders take time to fully develop.

Splenectomy considerations:

Comorbidities (eg, serious cardiopulmonary disorders) increase the risk of general anesthesia and post-surgical complications. A higher rate of surgical complications and a lower response rate is reported in most studies of patients older than 45-70 years of age and in those with various secondary forms of ITP. Care must be taken when considering options for patients with a history or serious risk of thrombosis.

At one end of the spectrum, splenectomy has the highest cure rate and may therefore be the preferred option for younger patients, who have the best response and lowest complication rates, engage in physically challenging sports or professions, are potentially noncompliant with protracted daily treatment, and do not wish to continue to “deal with” their ITP. Recommendations would be influenced by the results of platelet isotopic distribution studies (if confirmed and if available).

At the other end of the spectrum, we try to avoid splenectomy in patients over 65-70 years of age (depending on their physical condition) not only because of higher complication rates but also
because of lower response rates. The same considerations apply to the very frail, those with significant surgical comorbidities, history or risk of thrombosis, those with obligatory exposure to malaria or babesia, or those who have secondary ITP. Questions may be raised about post-splenectomy infection in teachers, veterinarians, health-care providers, travelers to certain areas, or others with increased exposure to infectious conditions.

Sandi
I think strong consideration needs to be paid to the possibilities of post surgery portal vein thrombosis. In our daughter's case she has needed eight different interventions since her unsuccessful splenectomy. It seems there is a greater risk when the surgery is preformed orthoscopically
Our Daughter now injects herself weekly with n-plate and daily with arixtra ( blood thinner ). If there was a balancing act before regulating her platelets, now add to that regulating blood thinner at the same time. So far they've not been real successful.
All this from a failed splenectomy.

A Clow, from what I remember of your previous posts, your daughter probably has familial thrombocytopenia and not ITP therefore I do wonder why they went the splenectomy route as splenectomies don't work with familial thrombocytopenia. Lots on the web about it, have you looked into it?

A Clow:

I do, quite often, mention the risk of blood clots/thrombosis following splenectomy. Not just right after the surgery, but it can occur even years later. I try to make everyone aware of it prior to the surgery. It's a fine line to walk though because I don't want to scare people all the time and be the voice of doom and gloom. I am seeing more clotting problems now though than ever before.

I'm sorry this happened to your daughter. It can be quite a juggling act to manage both at the same time.

Really good article!

Ellen (wife) was diagnosed in April with ITP, her lowest count 26k. She was responsive to pulsed dexamethasone (but short-lived) and got good results from IVIG - except for a long hospital stay for what turned out to be chemical meningitis! Her post-IVIG count has gradually dropped from a high near 300k down to below 90k.

We have done our homework, and Ellen is prepared to live with a moderately low count without treatment, and make every effort to avoid splenectomy, at least for a year, and probably indefinitely. We believe that better treatments are coming, but a splenectomy is forever.

Her hematologist wants her to get immunizations in case she needs a splenectomy. I see a battle on the horizon.

Questions:

• How do you decide what is "too low" to continue a wait-and-see approach?
• Is a splenectomy ever necessary if there are other treatments that have worked,
  or that haven't been tried, and aren't contraindicated?
• Are "emergency" splenectomies for extremely low platelets ever necessary,
  short of major bleeding? with major bleeding?
  
• Are the immunizations a good idea... just in case?
  is there any harm in having them?

Rob:


How do you decide what is "too low" to continue a wait-and-see approach?
Most ITP specialists will agree that counts of 20k to 30k are considered safe for most people. Unless there are bleeding symptoms, anything at or above that level should be okay for most individuals.

Is a splenectomy ever necessary if there are other treatments that have worked,
or that haven't been tried, and aren't contraindicated?
No, a splenectomy is never necessary if other treatments work, unless the spleen is enlarged (which is not likely with ITP).

Are "emergency" splenectomies for extremely low platelets ever necessary,
short of major bleeding? with major bleeding?
I think emergency splenectomies (or planned ones, for that matter) are rarely ever 'necessary'. With the TPO's that are available now, treatments actually have a better chance of getting platelets up than a splenectomy does. Unless the spleen is abut to rupture, there is no emergency. Just having low counts does not qualify as an emergency unless there is severe bleeding, and that does not mean that a splenectomy will automatically fix that. As one ITP specialist states, "We are not finding that people are dying from NOT having a splenectomy".

Are the immunizations a good idea... just in case? Is there any harm in having them?
Immunizations are a must prior to splenectomy. As for having them 'just in case', that is a judgement call. Sometimes the vaccines can exacerbate ITP since anything that stimulates the immune system can have a negative effect. Some people do just fine, others do not.

Sandi

What would we do without YOU!! So many of the questions in the back of my mind are answered by you!!

THANK YOU!!

Cindy Ann