Liver/abdomen pain

Hi everyone, I have been suffering on and off for at least a year with pain just below my ribs both sides.  I had an ultrasound of my liver last year which showed it was very enlarged and my haematologist told me it was enlarged because my liver was trying to make foetal haemoglobin.  I would regularly have a pre transfusion hb of 7.  I have tried increasing my transfusion regime and am trying to keep a pre transfusion hb of 9 but it is often difficult to get the staff to order me enough blood.  I am so sick of trying to explain the reasons for the need for a higher hb.  Over Christmas my liver was very painful and visibly enlarged and I later found my hb was 6.7, the pain went away after transfusion.  Has anyone else experienced this? And does anyone else get pains in the upper left abdo following blood transfusion.  I'm sure my spleen is enlarged too but my haematologist tells me he can't feel it.  I suspect this is because my large liver is covering the spleen, I'm sure post transfusion I can feel it somewhere just above my navel.  My ferritin has dropped significantly this year from around 6000 to 2900, it's been a long slog but am slowly getting to the goal of less than 1000 Do you think I ahold be more concerned with the liver pain or trust that it is simply the low hb which has caused the pain.  My main concern is that my liver is full with iron but I won't be sent for scanning until my ferritin is less than 1000 at least.  Thanks for any replies x

What is happening is called extramedullary hematopoiesis (EMH) and it is caused by the body's attempts to compensate for a lack of hemoglobin. The best way to eliminate this is by maintaining a higher hemoglobin level, so you will have to continue to to try to convince the staff about the importance of having a higher Hb level. It isn't accurate to say it is the result of trying to create fetal hemoglobin. If only that was the case, these masses might provide something positive, but they simply produce the same bad red blood cells that your bone marrow also creates when your Hb is low. The masses that grow in EMH may be harmless, but often they can cause serious problems if they put pressure on the spine or lungs. The drug hydroxyurea, which is used to raise fetal Hb in thals is also used to reduce the size of the masses. I would suggest talking to your hematologist about the masses and seeing if an MRI may be in order to accurately assess the size and location of the masses to see if any therapy like hydroxyurea would be indicated. Hydroxyurea may also be a consideration because you have not been able to get enough blood to maintain a proper Hb level. If it works to raise your Hb a bit, you won't be getting the iron involved in more transfusions and you may be able to make quicker progress in getting your iron load down. You've made good progress and what we typically hear from patients is that once the ferritin has made a good drop, there has also been a corresponding drop in the organ iron load.

Thanks Andy, I am grateful for the information and advice.  I will definately make a stronger effort to increase the pre transfusion hb.  I may have to be a little more forceful with the staff and insist on more blood.  I hope to have my ferritin within the target range in the next few months as the exjade is working very quickly now.  I previously took IV desferal for a number of years however due to repeated systemic infections this is no longer possible.  Once my ferritin is in range I should be eligible for MRI scanning but up until now my haematologist has been reluctant to do this as he says it is definate I will have high iron stores and that my only focus right now is to push on with the chelation.  He would prefer to wait until the ferritin is lower so that we can the use the results to work out a new chelation regime dependent on the stores.  He believes that MRI is irrelevant whilst my ferritin has been so high.  I will certainly research hydroxyurea to see if this is an option.  Can I ask, will the EMH masses ever disappear with transfusion therapy alone?  I have noticed that the visible hepatomegaly and pain is most often pre transfusion and it usually dissipates post blood.  I am grateful for the reassurance that the EMH should not be causing any liver damage but willtake heed of the warning about the lungs and spine.  Thanks  again for your knowledge and time, it is good to know that a second opinion is in agreement with my doctor!

Yes the masses can shrink when there is an adequate blood supply. Hydroxyurea would be better at this.

I want to point out that the purpose of the MRI has nothing to do with iron in this case. That can come later. An MRI now would be to determine the size and location of the masses, and whether or not action should be taken now.

hi
 also the pain can be liver-infarkt too.
 i had them a few years ago too.

Panos,

my son has terrible pain in his abdominal area and in the area of the liver.    The doctors say that they cannot find what causes the pain. They did a CT and an MRT but they only solution they found was to put my son on very strong pain medication such as targin which an opiate drug and highly addictive. The pai is still there.

How is a liver infarkt proven? or diagnosed?

hi georia

unfortunatly i havent the time to answere ur e-mail,
but i will the next few days....there is a lot i can told
u about the situation of ur son.
i have the same "Extrameduräre blutbildung" in my
body like him. i have this for over 20 years now.
and for the liver-infarkt...a good doc sees it by the
"Ultraschall" nowadays where i have the iron troughn
out of my liver the pain is gone away....
u must now i come down from 16.900 ferritin to 400 now.
and this is the key to a lot of our thalproblems.
gerogia please give me ur phonenr. or call me at 06184/55049.    panos