Spleen removal

Hello, everyone! I have a question for Andy and those of you who may have any kind of information to help me out. For those of you who know me, hello and miss you! For those of you who do not, hello and look forward to getting to know you! I am Danielle, also known as "Dee." I'm a Thal Major and receive two units of blood every two weeks, and still average an 8 hgb. My doctors are giving me two weeks to research spleen removal and to give them reasons why I shouldn't have my spleen removed, because they want it out. Now, I'm not really against it, I'm just nervous. Can those of you who have had your spleen removed tell me if it was a good choice, and/or what complications (if any) that you have had since its removal? I've been told by many Thals that it was a great choice, and I have also heard it was a bad choice because of blood clots and infections. I would also like to know if not having a spleen lowers the chance of being a candidate for possible cure with gene therapy in the future. I need all the info that I can get, because my doctors are really pushing this now and I need to give them my answer when I go back for blood in two weeks. I don't know why they are giving me such a short amount of time to give them an answer and what repercussions I will have if I tell them "no" so I need as much help as I can get. They feel that my quality of life is being compromised now because I sometimes go for blood every week for a "boost" and I'm there too often. (I honestly think that they just don't want me there as often). Please help! Thank you, all. <3


~Danielle

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Hi Dee,

The new Standards of care Guidelines for Thalassemia have been released. I will be updating our own document with this information. Please make certain that your doctors are following the guidelines concerning splemnectomy. My own opinion is that splenectomy should be a last ditch effort.
http://hemonc.cho.org/thal/documents/SOCguidelines.pdf

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. Often, hypersplenism develops because of
a low pre-transfusion hemoglobin. Increasing the pre-transfusion
hemoglobin to between 9.5 and 10 may reverse hypersplenism.
If a decision to perform surgery is made, partial or full
splenectomy is the option. Partial splenectomy is a complicated
surgery utilized to preserve some splenic function. It should be
reserved for infants requiring splenectomy. Full splenectomy
can usually be performed by laparoscopic technique. However,
open procedure is necessary in cases of marked splenomegaly.
The indications for splenectomy in hemoglobin H–Constant
Spring patients are different than in beta-thalassemia disorders.
Transfusion-dependent infants with hemoglobin H–Constant
Spring respond rapidly to splenectomy but require prophylactic
anticoagulation because of a high incidence of serious thrombosis.
Patients must receive adequate immunization against Streptococcus
pneumoniae, Haemophilus influenzae type B, and Neisseria
meningitides prior to surgery. Splenectomy should be avoided
in children younger than five years because of a greater risk of
fulminant post-splenectomy sepsis.
After splenectomy, patients should receive oral penicillin
prophylaxis (250 mg twice daily) and be instructed to seek urgent
medical attention for a fever over 101º Fahrenheit.
Post-splenectomy thrombocytosis is common, and low-dose
aspirin should be given during this time. Another complication
following splenectomy is the development of a thrombophilic
state. Venous thromboembolism, more common in thalassemia
intermedia and hemoglobin H–Constant Spring, can develop
following splenectomy.
Patients should have annual echocardiographic measurement of
the pulmonary artery pressure to monitor for development of
pulmonary hypertension.

Andy, thank you so much. This helps me a great deal. I am going to print this out and show it to my doctors. I am just not comfortable losing my spleen yet, especially if it's not significantly enlarged. My iron level is low enough (500) to not worry about and there is no other dire need to remove it other than getting transfused frequently. Thank you, again!


~Danielle

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High blood turnover is usually related to antibody reactions, which do normally pass, so this alone should not be considered reason for splenectomy. My opinion is that in most cases, splenectomy should not be done unless there is enlargement of the spleen to the point where it may be dangerous, as was the case with Nur, whose spleen was around 25 cm.

That's what I said but they aren't having it. My spleen is only about 14cm. They are really pushing this on me.


~Danielle

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Time to get a second opinion. Would it be possible to be seen at one of the thal centers like Boston or Philly?

I can probably schedule an appointment and take the trip there, but I don't even know where to start. Who are the specialists over there and how do I contact them? Plus, I don't know why I'm being given two weeks to decide. Sounds kinda weird to me. They're making it sound like they will stop treating me or something if I decide against them. Very weird.


~Danielle

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Dee,

Have you talked to Eileen at CAF? She may have some advice on how to handle the doctor's attitude. She could also help arrange a referal.

Yes, I actually have. My mother talked to her as well. It was a while ago though so I am going to contact her again with the recent ongoings.


~Danielle

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I hope you will keep your spleen Danielle.  Hypertransfusion will help shrink it. 

Sharmin

Danielle,

As discussed above, a second opinion is warranted. However, I would print the info from Andy and try one final time to convince the existing doctors. Yous spleen is not enlarged, iron level is good, the only factor your doctors are considering is the frequent blood transfusion. Please research and take your time to decide, I would make it clear to my doctors that I respect them but need more time as I am working on it.

Good Luck and please keep posted.

While I know a lot about spleen and that docs says I have very minor spleen enlargement which isn't even noticed by me or any docs, most of the times.

My questions are:

What actually increases the spleen size?
What could be the symptoms of it other than the stomach skin increase?
Any ideas or possibilities that it would come to normal size in future? If so, how? Making iron levels normal or increasing blood transfusion rate?

Thank you. :-)

Hi Andy,
            its  my  liver  and  spleen ultrasound  report
       
LIVER     
        size:    14.6
        texture: normal

Hi Andy,
            its  my  liver  and  spleen ultrasound  report
       
LIVER     
        size:    14.6
        texture: normal
        mass effect: none
       hepatobillary  radicles  and casculature: normal
       portal vein: 1.0
   
Spleen
        enlarged
         size:  16.5x16.5x8.5
Pancreas
         normal  pancreaseses  is  seen SP. Vein: 0.8
Lymph  nodes: none
Remarks : spleenomegaly
  komal

Your spleen is enlarged, but not to the point requiring removal. The best way to reduce the size is by maintaining an Hb level of no less than 9.5-10.