How to reverse hypersplenism and iron overload

Friends, I am in catch 22 and need your advice. I weigh 51kgs and it's been 4-5 months now that my hb is unable to maintain pre-transfusion levels even after  getting 2 units every 3 weeks whereas upto last year I used to get 2 units every 4 weeks. For example, my last pre-hb was 8.8 and after 2 units transfusion and 20 days my Hb is 8.2. The gap is reducing which is alarming for me as it appears that spleen is becoming hyperactive. My platelets are in 70-80's range and spleen is upto belly button now. Docs here won't give more blood because ferritin is in 5000s and I am on 24/7 chelation . I want to stop spleen getting overactive and I want to reduce Iron at the same time.

Shall I get more units transfused now that may increase ferritin by extra 1000/2000 to maintain pre-hb levels of 9.5 and continue chelating after that - OR- is it better to get as fewer transfusions I can, pre-Hb of 8-8.2, and get the ferritin in atleast 2000s before increasing the blood requirement?

My worry is that doing the latter option will make my spleen bigger and bigger and take me to the point of splenectomy that I want to avoid at all costs.

Another question is that in how many months of low hb does the spleen starts getting larger? Does it gets big over 1 or 2 months?

Please advise.

Waleed,
Whats ur spleen size and blood transfusion volume ?

maintaining 9.5gms% will not work for hyperspleenism , hb must be above 11 gms% pre transfusion to put a check on hyper spleenism.

Regarding higher blood tx , high fe, low hb , we can call it a trap because spleen destroys excessive RBCs consequently u need more tx, it increases ferritin (and inspite of 24/7 chelation fe will not decrease).
In addition, your platelate are low might be because of hyper spleenism.
 

Hanumant,

Exact size isn't known but it is clearly felt upto belly button while.lying straight. Weight is 51 kgs and tx 2 units of 250-300mg(not weighed exact) every 3 weeks. Yearly tx volume doesnt fall under excessive criteria but what is happening from past months is clearly indicating faster hb reduction. In the guidelines it is 9.5-10g/dl to reverse hypersplenism.

Glad that I met my old Dr. at the clinic and he will now be seeing me which is a good news. Doc mentioned that we will have to increase hb upto 9.5-10 but that will be done gradually because if he transfuse more blood at once the spleen will destroy blood even faster and get bigger. Anyone experienced in this?

Doc also advised me to restart deferiprone (instead of exjade) with desferal as he believes the low platelets aren't because of kelfer but its due to spleen. I am thinking to incorporate deferiprone in my existing combination therapy. Have anybody tried desferal/exjade/kelfer all at once together? (i know Panos used them on alternative days)

I know that guidelines reco 9.5 - 10 gms % hb but there is a few chance that it will work for you.
The both thing you asked i had been through i.e. hyper transfusion because of spleen and all the 3 chelators simultaneously.

Remembering Panos,
I would like to repeat DONE THAT BEEN THERE.

I know that guidelines reco 9.5 - 10 gms % hb but there is a few chance that it will work for you.
The both thing you asked i had been through i.e. hyper transfusion because of spleen and all the 3 chelators simultaneously.

Remembering Panos,
I would like to repeat DONE THAT BEEN THERE.

Dharmesh what I read on previous posts is that Panos used 3 chelators but not all three at the same time but rather on alternating days. Did you take all 3 chelators simultaneously? Did you start increasing your blood intake gradually? How fast (in days/months) the spleen grows rapidly? In your experience, what should be the decisive point to finally agreeing on splenectomy? Share your experience.

Brother Waleed, previously after transfusion, was two units always enough to raise your Hb levels to a good level?
What Hb level did you work to achieve after transfusions..
Did you feel tired more often...

We try to achieve Hb level at 15 after transfusions. This has always helped my sons with energy levels, growth, and with good chelation healthy organs and happy ferritin levels, Alhumdulillah.

just recently with things being changed to being transfused 'units' rather than 'mls' needed per body weight, my son's Hb level has been dropping too. Alhumdulillah, spleen is ok.

Brother Waleed, please step up the chelation programme. Desferal PLUS Ferriprox/Deferiprone is an excellent combi chelation programme...seven days a week. You will definitely see the ferritin dropping, in shaa' Allah. Perhaps this will help settle the spleen too.
I think, though I'll need to check notes, that my younger son's spleen was mildy enlarged but when his ferritin came down then the spleen reduced to normal.

I pray that it works out for you.


 

After reading other posts (thank you) I think the brothers spleen, by Allah's will, may be healthy because they mostly aim at Hb 15 post transfusion.

....so this means we need to fight for 'no change' in their transfusion treatment plan more to keep it the old way to maintain spleen health too..and not to agree to transfusing so that they are short of blood every transfusion.

 

Dharmesh what I read on previous posts is that Panos used 3 chelators but not all three at the same time but rather on alternating days. Did you take all 3 chelators simultaneously? Did you start increasing your blood intake gradually? How fast (in days/months) the spleen grows rapidly? In your experience, what should be the decisive point to finally agreeing on splenectomy? Share your experience.

Yes, i used simultaneously.
Keep monitoring the spleen status that only can be done now.
And follow the suggestion given in other post

Dharmesh,

Did you raise your Hb upto 10 to cool down spleen or you decided to go under splenectomy pretty early? At what rate shall one increase the transfusions? Go after 2 weeks instead of 3 weeks or keep the weeks same but increase blood bags?

I tried to improve the HB but the spleen was working very hard (Lol)

I was pretty late for splenectomy by 10 years

My tx duration was 1 unit 15 days which comes down to 1 unit 12 days then 10 days then 2 units every 14 days and still there was no improvement. At last , it was 10 days and 2 units.

I was done with all the measures to save spleen but only after removal i realized the benefits.

I tried to improve the HB but the spleen was working very hard (Lol)

I was pretty late for splenectomy by 10 years

My tx duration was 1 unit 15 days which comes down to 1 unit 12 days then 10 days then 2 units every 14 days and still there was no improvement. At last , it was 10 days and 2 units.

I was done with all the measures to save spleen but only after removal i realized the benefits.

hello,

Im Thal Major and facing the same issues. My spleen is enlarged, having 2 units transfussions every 10-14 days, cbc too much disturbed and my ferritin is also high.
I think i nw need spleenactomy, but my Dr is reluctant.

I dont understand what to do?

Asma,

I'm sorry to hear about your current condition however avoiding splenectomy were possible is generally the best advice.

I am assuming the reason for your regular transfusions is low hgb however have you researched Hydroxurea or discussed the new upcoming and trial drugs with your haematologist for Thalassemia in general?

With regards to your ferratin, this isnt an entirely accurate way to check your iron levels. Ask your haematologist to do a Iron Serum blood test as well as a Ferriscan to see iron build up in the liver. There is also a similar scan to check iron levels in the heart. The Ferrisacan is none intrusive and is simply an MRI were you lie down and a machine scans over you and checks to see the concentration of Iron in the Liver. These are far more accurate tests to diagnose iron levels.

p.s. when you say your spleen is enlarged, how large is it exactly? have you had an echo of it so we can check numbers?