extramedullary hematopoiesis around the spinal cord

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Offline Lilian

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extramedullary hematopoiesis around the spinal cord
« on: June 13, 2008, 04:08:19 AM »
Hi all,

I did an MRI last year, to check on the iron deposits in my heart and liver. From the MRI, the radiologist alerted my hema that i have a mass around my spine. I followed up with a CT scan, which showed extramedullary hematopoiesis around the spinal cord.

I don't really know what it means, except that my doc explained that it's due to my bone marrow's over-activity. I was on hydroxyurea but it crashed my Hb level, thus was taken off it. Now, i'm on more frequent transfusion. fortnightly.

i was told that only treatment for extramedullary hematopoiesis is radiation?? but this is some drastic measure my doc do not want to explore yet, as it may lead to the damage of other good cells. I dunno.... all this is very confusing. is the radiation supposed to be something like chemotheraphy?

my doc said to just monitor the mass, and to look out for back pain, which may be caused by spical cord compression.

what are the outcomes for extramedullary hematopoiesis ? is the only way out radiation? what are my options? my frequent transfusion is also putting stress on my spleen, which is very big. but doc have not seen the need for a splenectomy.

is extramedullary hematopoiesis common amongst thal majors? any advice? thanks in advance :)

cheers,
lilian
« Last Edit: June 13, 2008, 04:14:42 AM by pastel_lilian »
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Offline §ãJ¡Ð ساجد

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Re: extramedullary hematopoiesis around the spinal cord
« Reply #1 on: June 13, 2008, 04:44:48 AM »
Hi Lilian,

Extramedullary hematopoiesis is the body's counter measure to correct the Anemia. Normally our body makes most of it's blood from the bone marrow within the bones. When the bone marrow is unable to keep up to the demand of blood (because of our Thalassemia) then other organs like the liver and spleen (which have the ability to make some quantity of blood) also dig in to make blood when required. Now when these are over stressed too then more mass around these organs starts to grow for further Extramedullary hematopoiesis. These extra masses can form around spleen and even the vertebra column; causing stress in abdomen or spine.

I guess your doctor wants to remove them by radiation.

The best way to prevent this from happening again is to keep your blood levels in check so that there is no need for Extra Medu. Hemat.
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Offline Nur

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Re: extramedullary hematopoiesis around the spinal cord
« Reply #2 on: June 14, 2008, 03:49:13 AM »
hi,

I have been diagnosed with Extramedullary hematopoiesis due to the lack of transfuison. My doctor is trying to prevent from radiation therapy by keeping my HB above 8. This is to prevent the bone marrow from overworking and hopefully the 'tumor' would reduce in size overtime. She did mention that it might get reduced naturally but the chances are too slim. The best way is to keep the HB level up so it does not grow bigger. The last option that she want to avoid is radiation therapy.

I do not know much about the therapy but i do know that the tumor can be wiped out by radiation. so does other good living cells. In this case, i dont want to be radiated yet! Or else...I might change my name into Super Radiation Girl!   :biggrin

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Offline §ãJ¡Ð ساجد

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Re: extramedullary hematopoiesis around the spinal cord
« Reply #3 on: June 14, 2008, 04:25:27 AM »
:hmm

Maybe you'd get hematopoietic super powers. Zap anything with your power and a fountain of blood starts to erupt.  It would be quite horrifying but would solve the blood availability issues :biggrin
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Offline Manal

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Re: extramedullary hematopoiesis around the spinal cord
« Reply #4 on: June 14, 2008, 12:14:09 PM »
Hi

I know a little girl ( 5 years ) who is a thal major na d transfuse every 25 days and she had extramedullary hematopoiesis near the lungs, but the doctor prescribed her hydroxyurea (hydrea) in adddition to avoid having a Hb of less than 8......... hope this helps

manal

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Offline Andy Battaglia

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Re: extramedullary hematopoiesis around the spinal cord
« Reply #5 on: June 14, 2008, 06:43:41 PM »
From http://www.appliedradiology.com/case/case.asp?Id=606&IssueID=154&CatID=43&SubCatID=101&ThreadID=&Quiz=

Quote
Extramedullary hematopoiesis is the production of blood elements outside the bone marrow cavity...Extramedullary hematopoiesis occurs in severe chronic anemia, which can arise from deficiency disorders such as vitamin B12 or folate deficiency2 or from disorders affecting pluripotent stem cells. The latter are classified as: a) stem cell dysplasia, as in thalassemia, sickle cell hemoglobinopathy, and hereditary spherocytosis...Extramedullary hematopoiesis occurs as a compensatory mechanism for abnormal hematopoiesis when normal red marrow is unable to function because of deficiency disorders or because of various pluripotent stem cell disorders. Most often, it is microscopic and asymptomatic but can sometimes produce organomegaly, tumor-like masses and even life-threatening symptoms. Extramedullary hematopoiesis should be considered in the differential diagnosis in a patient with chronic severe anemia and a paraspinal mass.

Basically, Extramedullary hematopoiesis is the reaction of the body to a low hemoglobin state. Because the bone marrow is not producing sufficient red blood cells, the body tries to compensate by growing red cells elsewhere. This is usually at the cellular level, where it normally is not diagnosed, although it is known to show up on some scans, where it may be misinterpreted as lymphoma because the reaction is identical in the tests. This can potentially lead to treatment for lymphoma that doesn't exist in the patient. Extramedullary hematopoiesis becomes a problem when it grows as a mass in certain locations, like against the lungs, as with Courtenay, or against the spine, as with Nur and pastel_lillian. If the mass grows large enough, it can cause serious injury to the spine. By the way, Courtenay is a thal minor, so doctors everywhere should take note and throw out the books on this. Extramedullary hematopoiesis occurs in thal minor, intermedia and major. The books will tell you that it is found in intermedia but we can't live by books. In our real world, extramedullary hematopoiesis does often occur in majors and I suspect, also in many minors who live with low Hb.

As Sajid and Nur have stated, the first line of treatment is keeping the Hb level up. Nur's problem developed because she has difficulty finding matched blood and often her Hb drops to very low levels. This troubles me greatly, as Nur is a mom (who had her child without any hormones or help, so yes, it can happen), and also one of my dearest friends. Her doctor's strategy is to try and keep her Hb up so the mass doesn't grow any further. If it does, she most likely will require radiation treatments to shrink it. Having talked to people who have had radiation for different cancers, I will say that it isn't as bad as one might imagine, so if it becomes the only choice, please have some confidence because the alternative of doing nothing can actually lead to death.

There are two other ways that extramedullary hematopoiesis is dealt with. One is surgery to remove the mass. This can be dangerous if the mass is pressing on the spine. The other treatment is more experimental and has been mentioned here in this thread and also earlier at http://www.thalassemiapatientsandfriends.com/index.php?topic=1207.msg12515#msg12515   
Hydroxyurea is used to increase Hb which will also suppress the further formation of the extramedullary hematopoiesis. This is somewhat experimental but it may have application outside use with intermedias, just as raising Hb in majors with hydroxyurea may have some application.

You should ask your doctor about hydroxyurea and if it would be appropriate to try in your case. You should also prepare yourself mentally for the prospects of having radiation treatments. If the growth gets too large and endangers your spine, there will be little choice.

Andy

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Offline Nur

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Re: extramedullary hematopoiesis around the spinal cord
« Reply #6 on: June 15, 2008, 02:20:23 PM »
hmmm... :hmm hematopoietic super powers.... what costume should i wear... :biggrin I, hematopoietic super powers to the rescue! to fight off iron man  :grin

From http://www.appliedradiology.com/case/case.asp?Id=606&IssueID=154&CatID=43&SubCatID=101&ThreadID=&Quiz=

Nur's problem developed because she has difficulty finding matched blood and often her Hb drops to very low levels. This troubles me greatly, as Nur is a mom (who had her child without any hormones or help, so yes, it can happen), and also one of my dearest friends.

Andy,

Your words brought tears to my eyes as i don't have that many friend who really cares about me and you are always there to cheer me up. So far, since i'm getting my bloods from Penang i don't feel the pain at all (except once which is not as bad as usual). So hopefully keeping the HB level up is doing some good to me.

The word radiation sounds terrible and i really don't know what happens afterwards to the body. I felt relief when you said it's not that bad.

I have asked my doctor about hydroxyurea but something in my blood test show that it is not suitable for me to try it.

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Offline Lilian

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Re: extramedullary hematopoiesis around the spinal cord
« Reply #7 on: June 16, 2008, 09:51:15 AM »
Hi Andy,

Thanks for your information, advice and encouragement. YOu're trully a blessing to us all.  :hugfriend

If things come to a stage where radiation is the only option for me, don't worry; Bring it On, i'd say.

 My parents have sacificed so much and i've crossed so many obstacles, making me the person i am today. what's a little radiation compared to erasing all their hard work?

Thanks to everyone for your kind advice too.

Nur, I do hope things will work out well for you. monitor that bugger of a mass, and keep me updated yea? take care  :hugfriend

cheers,
lilian
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Offline Bobby

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Re: extramedullary hematopoiesis around the spinal cord
« Reply #8 on: June 20, 2008, 05:44:15 PM »
Last year I found out that I have extramedullary hematopoiesis around my spine.  They only found it because I had an MRI on my kidneys.  So far my Dr. hasn't mentioned doing anything about it. He just said if you start to feel numbness we might have to treat it with radiation therapy.  I thought it was pretty cool, It's like when a lizard grows a new tail.  :biggrin

Wishing you all well.

Bobby

 

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