Splenectomy(removal of spleen) at an early age or when the spleen gets enlarged?

hello  Manai,

thankyou for your question ,

they doctors are unsure whether i can have the operation or not, probably not.
However , they want to decrease my chance of iron overload (from happening again )
and to decrease the transfusions from every three weeks to perhaps every 4 or 5 weeks
and to increase my HB

Do you think if the spleen is taken out these above things will happen ?
any information you have on this matter would be much appreciated

thankyou

lillie

Hi Lillie,

To my understanding,some patients do observe increase in transfusion intervals after splenectomy,even some can get transfusion free,like our member Lyanne is,but same can not be said for everyone,every person's body works in different way,so it's not confirmed how your body will react to splenectomy.
One more thing i've learnt from the forum is that having splenectomy can increase your chances of getting infections,you'll have to be on antibiotics.

Good luck.

Zaini.

Hi Lillie

I have learnt in the Singapore conference that splenctomy is not advised as long as you can live with your spleen and it does not threaten your life like becoming very big in size.

But as Zaini said, taking the spleen could result in increasing the intervals between transfusions, but in the majority of cases the usual frequency before splenectomy is back.

Also taking the spleen off increases chances of infections and thrombosis.

I guess that in your case, keeping the spleen out weighs taking it off, first because the surgery might be dangerous ( since the doctors couldn't decide till now and of course they are the only ones who have a say on this). Second because the target is to decrease the iron overload and i guess that you can achieve this by having compliance to chelation ( if your are not dedicated to chelation in the first place, you will get iron overload even if you removed the spleen), third, decreasing the transfusion rate is not guranteed

Lillie, i am sure you understand that this is not a not a medical advice, but is just my opinion

hope it helps

manal

We have to remember that we are talking about Congenital dyserythropoietic anaemia and not thalassemia. Treatment protocols cannot be assumed to be identical with thalassemia treatment. This is a very rare condition and due to the small amount of patients, treatment protocols have not been as well developed as with thal. However, splenectomy is often used as part of the treatment. A study in 2006 found the following in relation to CDA I

http://bloodjournal.hematologylibrary.org/cgi/content/full/107/1/334

Splenectomy was performed on 7 patients ranging in age from 9 to 28 years. Data before and after splenectomy are available in 6 of those patients. Transient increase of hemoglobin concentration was seen in 2 cases, but was not different before and after splenectomy for the group as a whole (Wilcoxon signed rank test, P = .44). Improvement of quality of life was not documented. The sister of one patient (case not included in this series) died one month after splenectomy of "hemolytic anemia." All splenectomized patients had long-standing thrombocytosis with maximum levels between 450 and 830 x 109 cells/L (median, 780 x 109 cells/L). Two had splanchnic or major deep venous thrombosis. Further increase of ferritin after splenectomy was documented in 4 of the splenectomized cases and all had to be treated with deferoxamine...
In contrast to CDA II, splenectomy is not recommended as a standard procedure even in patients with marked anemia, although in exceptionally severe cases it may lessen the need for transfusions.39 Our observations are consistent with the only report describing long-term follow-up in one patient.32 As in CDA II,33 splenectomy does not prevent further iron overloading. Interferon-alpha was effective in 5 of our patients and in all cases reported.5 According to a recently published follow-up of the first patient treated with interferon {alpha}-2a,40 this treatment, beyond normalizing the hemoglobin concentration, seems also to normalize the up-regulated enteral iron uptake, responsible for the iron overloading in CDA as well as in other states with chronic ineffective erythropoiesis.41

In CDA II splenectomy does appear to have some benefit.

http://www.enerca.org/Portals/0/pdfs/Newsletter_June_2007.pdf

In CDA II, splenectomy leads to a moderate and sustained increase in hemoglobin concentration and decrease of serum bilirubin levels, as shown in 48 patients from the two registries [5; 11] and in 41 patients published as case reports. If measured, shortened red cell survival normalizes [1], demonstrating that, as in hereditary spherocytosis, abnormal CDA II erythrocytes may survive normally in an asplenic individual. No overwhelming bacterial infections were observed after splenectomy.

Even with thalassemia, splenectomy is not as widely accepted as previously and is mostly recommended in cases of hemolysis or gross enlargement. Splenectomy as a therapy for thalassemia is basically a shot in the dark. Sometimes it helps to reduce transfusion frequency and sometimes it doesn't. Removal of the spleen has a detrimental effect on the immune system, so it should be carefully considered before choosing it as a strategy. The type of CDA is important here and since we are dealing with a variant, it is probably not going to be within our expertise to  make any recommendation.

Hi, my name is Vidi from Indonesia, I have a 5,5 years daughter who diagnosed with betha HbE thalassemia when she was 10 months old. In the first year,she gets blood tranfusion every 3 months, and now every 3 weeks. I want to know what kind of thalassemia than my daughter have?? Is it intermedia or major??
My daughter,ayudia, also have a very big spleen (S VIII), her doctors says that she need to have splenectomy. Is anybody can help me give the name of the doctors and the hospital in Malaysia or Singapore that have a experiance to this procedures?? Because I can not found one in Indonesia..

Hi Vidi ,
  to the Family , i hope you willl find the answer of your question realy soon , i cant say anything bcoze i m not an expert over it , i hope that, Admin of this site and some senior members will reply to your question with helpful information ... please keep in-touch and you will get your information related to your concerns very soon ... keep posting bcoze its very nice to have you here ... Wish you, your family and especialy to your daughter all the best of luck  ...

Best REgards
Take Care
Umair

Hi Vidi,

Your daughter is HbE beta thal. The condition varies from intermedia to major depending on transfusion needs. Your daughter's is like thal major.

We have some listings for Singapore care in out treatment centers section. http://www.thalassemiapatientsandfriends.com/index.php?topic=65.0

Please get an opinion from one of those hospitals before deciding on splenectomy.

Vidi,

HB ß E Thalassaemia varies widely and needs to be treated as per its clinical presentation - it can go from moderate anemia to transfusion dependent anemia (similar to ß major).

Since your daughter is only 5.5 years, and is on a 3-week transfusion schedule, I would agree with Andy that your daughter is more likely the latter.

However, again since she is only 5.5 years age, please ask the doctor whether there is a likelihood that a splenectomy could remove the transfusion dependency. Do keep in mind, though, that there are no guarantees on this - the doctors can only give you a likely outcome.

Poirot