Is a spleenectomy warranted? My case

Hello everyone. My doctor is suggesting I have a spleenectomy done. I'd like to mention every doctor I've ever had since birth has pushed me for spleenectomy since I was young. I'd like to know what everyone thinks about my situation and what recommendations you may have. Here are my facts:

25 years old, male
6 foot, 70 kg
transfusion requirement: 750 to 770 ml every 3 weeks since last 2-3 years. MAINTAINING hgb ~ 9.3
Previously maintained on 680 ml every 3 weeks BUT body weight was at that time: 59 kg
spleen usually non-palpable, occasionally felt to be 2-3 cm
Exjade 2500 mg oral daily
ferritin: 1100
T2* heart ~ 35 ms
Liver Iron content: ~ mild overload
Normal WBC, Normal Platelets, Completely normal Labs (exception of Hgb/Hct)
Slight elevation of bilirubin

Here's the story:
My doctor has been pushing me to have a spleenectomy done since my blood requirement has slowly increased from 680 ml to 770 ml now. When I used to receive 680 ml my weight was roughly 59 kg. I put on some weight and now weigh around 70 kg. I currently receive 750-770 ml to maintain the same hgb I maintained previously (~9.3ish) My ferritin has come down and heart/liver iron content improved over the past few years.

Does it look like I need a spleenectomy at this point in time? My doctor's argument is that 750 ml is too much blood and I should not need more than 2 units (680ml roughly) at a time (every 3 weeks). I am currently in opposition to a spleenectomy based on the reasoning that my WBC, platelets are normal and spleen is not grossly enlarged. Any suggestions?

Again we see a doctor operating by outdated information. Whereas splenectomy was once done routinely, it is no longer done unless there is a danger to the patient, either because of hypersplenism that cannot be controlled or due to excessive size. Study of those who have had splenectomy show that problems crop up later in life, such as thrombosis, which can cause life-threatening clotting. See http://www.thalassemiapatientsandfriends.com/index.php/topic,1762.msg14639.html#msg14639
The attached link is for the Standard of Care Guidelines for Thalassemia which are followed at the Centers of Excellence in the US.

4.5 Splenectomy
The use of splenectomy in thalassemia has declined in recent years. This is partly due to a decreased prevalence
of hypersplenism in adequately transfused patients.
There is also an increased appreciation of the adverse
effects of splenectomy on blood coagulation. In general,
splenectomy should be avoided unless absolutely indicated.
Splenectomy is indicated in the transfusion-dependent
patient when hypersplenism increases blood transfusion
requirement and prevents adequate control of body iron
with chelation therapy. An enlarged spleen—without an
associated increase in transfusion requirement—is not
necessarily an indication for surgery. Patients with hypersplenism
may have moderate to enormous splenomegaly,
and some degree of neutropenia or thrombocytopenia
may be present.
Annual transfusion volume exceeding 225 to 250 mL/kg
per year with packed red blood cells (hematocrit 75 percent)
may indicate the presence of hypersplenism. The
volume calculation should be corrected if the average
hematocrit is less than 75 percent. The possible development
of alloantibody should also be ruled out.
Splenectomy should be avoided unless there is an inability
to maintain iron balance with optimal chelation, or if
there are clinically significant complications such as pancytopenia
and marked enlargement.

For your weight, this means the amount of blood taken would have to be over 17,500 ml/year. Your intake is under 14,000. Do not let yourself be talked into unneeded surgery that has shown negative long term effects in thalassemia patients. Your ferritin is controlled and your spleen is small. There is absolutely no reason to recommend splenectomy, which in many patients, give only a short term reduction in the amount of blood needed.

Thanks Andy! This is exactly what I have mentioned to my hematologist. He refuses to believe this information and is certainly acting on old information. He doesn't acknowledge my weight increase and its correlation to my increased blood requirement. Which center is the best for Thalassemia care in New York? Would it be Cornell? To your knowledge how is the patient burden at that facility? Would they be open to new patients and do they provide a caring atmosphere? Thanks!

Andy would know more but we take our daughter to CHOP..Childrens Hospital of Philadelphia. They see a lot of thal patients and our always looking to help.

To Andy's point... DO NOT get the splenectomy...I am not a Doctor but probably have spoken with almost every "good" thal doctor in the U.S. and they are strongly against it.

James

Cornell is one of the Centers of Excellence. I would suggest talking to Eileen at CAF about a referral.


Cooley's Anemia Foundation
330 Seventh Avenue, #900
New York, NY 10001
(800)522-7222
Fax: 212-279-5999

Patient Services Manager
Eileen Scott
eileen.s@cooleysanemia.org

Addressing the subject of doctors not being up to date on thalassemia treatment, and why I stress annual evaluations at a Center of Excellence each year: Patients in managed care programs are routinely living into their 40's, 50's and even 60's. This is the case because an effort is made to stay current with advancements in treatment and understanding what that treatment should be. A doctor operating with information circa 2000 will not be aware of the changes in treatment during the past decade and will be doing his patients a great disfavor. I am very troubled by the number of patients in the US who are still dying in their 20's and 30's because there is no excuse for it anymore. I feel that every US patient should be registered with CAF and also be directed to a Center of Excellence for an annual evaluation, so that all the necessary tests and scans are done and so that information can be used to adjust the treatment regimens where needed. CAF is doing a great job directing patients to thal friendly doctors and it should always be the starting place when trying to decide where to go. And frankly, there is not a single doctor out there who isn't aware of the duty to always continue learning so that information is always current. Not doing so might let one skate along in terms of treating the common cold, but when it comes to thalassemia, it doesn't work. The amount of research about treating thal is phenomenal and we can expect significant advances over the next 25 years. Doctors need to stay current, and hematologists especially so.

My hematologist is primarily concerned with bone marrow transplant and stem cell transplant for oncology patients. That is is "money-maker." It is unfortunate that his Thalassemia patients have to suffer due to his lack of interest in this field. However, he would beg to differ.

One of my employees was pressured into a BMT to cure lymphoma after he had a recurrence in one spot in his body. 2 1/2 years later, he is alive but nearly died a couple times, spent a long time in the ICU, and still is dealing with the effects of having a unrelated BMT during his mid 50's. He recently told me that if he had the choice, he wouldn't do it again, but would opt for chemo. The BMT was far more expensive than chemo and I have always felt that was why he was pressured to go for a BMT. Don't allow doctors to pressure you into unnecessary procedures. Splenectomy now falls into that category for most thal majors.

One of my employees was pressured into a BMT to cure lymphoma after he had a recurrence in one spot in his body. 2 1/2 years later, he is alive but nearly died a couple times, spent a long time in the ICU, and still is dealing with the effects of having a unrelated BMT during his mid 50's. He recently told me that if he had the choice, he wouldn't do it again, but would opt for chemo. The BMT was far more expensive than chemo and I have always felt that was why he was pressured to go for a BMT. Don't allow doctors to pressure you into unnecessary procedures. Splenectomy now falls into that category for most thal majors.

great Andy sir,
you have command on each topic.