Hello to all

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Offline C3nZ0

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Hello to all
« on: November 27, 2006, 12:10:59 PM »
Hi, my name is Vincenzo (32 y) and write from Italy (I use google-traslate to help me to traslate, sorry  :wink)
I'm a Thal Int "light". About 3 mounth ago, I thought to be a Minor, but after genetic test has notice I have a double eterozigote (b-thal & alpha anti3.7).(there are anyone like me here?)

I don't had need of transfusions (my HB is over 12), but now I have problems with iron overload...  :mad

I have made not invade test to liver (like SQUID, http://www.ge.infn.it/~thalas/THALAS.htm), but the machine is experiences them the outcome is uncertain  :huh , therefore they have decided to made a "laparoscopia" for hepatic biopsy (a small surgical operation with cut, removal, and seam  :( )

For now is all, "Ciao a Tutti"

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Offline §ãJ¡Ð ساجد

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Re: Hello to all
« Reply #1 on: November 27, 2006, 01:00:49 PM »
Hi Vincenzo,

Welcome to the site! I hope you will find it very informative and all members very friendly and helpful. Please feel free to ask any question that you may have!

Take care, Peace!
اَسّلامُ علیکم Peace be Upon you
§ãJ¡Ð ®âµƒ
Web Site

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Offline Christine Mary

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Re: Hello to all
« Reply #2 on: November 27, 2006, 01:31:18 PM »
WELCOME VINCENZO!!!!

 :hug

Lauryn's Mom

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Offline Manal

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Re: Hello to all
« Reply #3 on: November 27, 2006, 02:48:25 PM »
Welcome Vincenzo.

I am sure you will like the site.

Why are you classified intermedia and your HB is 12?? Usually intermedias' HB ranges from 7 to 10??

Take care


Manal


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Offline C3nZ0

  • I'm thal Int, but minor trait
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Re: Hello to all
« Reply #4 on: November 27, 2006, 03:10:21 PM »
Why are you classified intermedia and your HB is 12?? Usually intermedias' HB ranges from 7 to 10??

Thanks to all for wellcome  :biggrin

My trait is like a minor (hb is higher), but I have globule red more instable because I have less Beta-chains (b-thal eterozigote) and more alpha-chains (triplication gene apha eterozigote); my body make some blod, but the iron into GR come to my liver...
This July my "FERRITINA" was about 1000  ??? (but I don't never transfusion).
I was very confused about it, in a lot of documentation that I have read my situation is still mentioned...
My situation is very light for now (and I hope that don't change)

The 2 last week, my doct confirmed me that I'm a Thal Int (very light).

PS i have some problem with medical eng. words ... sorry :wink

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Offline Andy Battaglia

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Re: Hello to all
« Reply #5 on: November 27, 2006, 03:25:10 PM »
Hi Vincenzo,

Your ferritin (iron levels) is high because you body is absorbing more iron from food than it needs. You should be on a low iron diet and should also be drinking tea with your meals, as tea can reduce the amount of iron that your gut absorbs from food.

This case is a perfect example of what we are talking about in our thread at

http://www.thalassemiapatientsandfriends.com/index.php?topic=562.msg4162#msg4162
    
Hemoglobin Variants: Are You Really A Minor?
Andy

All we are saying is give thals a chance.

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Offline Manal

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Re: Hello to all
« Reply #6 on: November 27, 2006, 05:02:38 PM »
Andy

Why is Vincenzo's body absorbing more iron though his HB is high? To my knwledge, iron starts to be absorbed in the guts as a result of low HB??  I can't understand???

Manal

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Offline Andy Battaglia

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Re: Hello to all
« Reply #7 on: November 27, 2006, 05:21:42 PM »
Manal,

That's a very good question. It may be one of several possibilities. His Hb may not be consistent at that level. It may have something to do with his odd gene structure, or even the blood that is being produced. The one possibility that should definitely be checked is if Vincenzo has hemochromatosis. This should be determined, as it is often not diagnosed and is much more common than previously suspected and can coincide with thal.
Andy

All we are saying is give thals a chance.

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Offline C3nZ0

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Re: Hello to all
« Reply #8 on: November 27, 2006, 06:40:35 PM »
My HB is "higher" but is however low (normally is over 14). This condition cause in me 2 problems:
1- I absorb more iron from my diet (like 50%; a normal people absorb lke 5%)
2- I habe a lot of Globale Red, but it life very less... therefore my body have some "GR died", and my liver try to clean my blody and clean up this GR whit its iron.

I have a "secondary hemosiderosis": is like hemochromatosis, but it isn't caused from genetic problems (I have readed a lot of documentation in italian languages about it)


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Offline Andy Battaglia

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Re: Hello to all
« Reply #9 on: November 28, 2006, 04:30:13 AM »
For those who are having a hard time following this discussion, I will try to summarize what Vincenzo has told us, and I do think this discussion fits right in with our discussion of hemoglobin variants and minor status. Vincenzo has both beta thal minor trait and alpha anti3.7, which is the most common of the alpha thalassemia traits. Alpha thal is the most common single gene disease in the world, found much more frequently than beta thal. Researchers are beginning to see that the combination of alpha and beta can produce problems beyond what would be seen with only one or the other of the types of mutations. This combination is causing chronic hemolysis, which is the premature destruction of red blood cells, in Vincenzo. Because of the loss of red cells (RBCs) his body is producing new red cells at a faster rate than normal, resulting in excess iron absorption in the gut, to feed those new RBCs. This has led to iron overload with a ferritin level of 1000. The doctor has suggested phlebotomies (blood-letting or taking of blood) as a treatment to reduce the excess iron. This is a routine treatment in hemochromatosis, and as we have occasionally heard here, has also been sometimes used as treatment for non-transfusing intermedias with iron overload. The advantage of this over desferal is there are no side effects or the inconvenience of desferal. The problem he is having with his liver is that the liver and spleen filter out these RBCs and the iron present in the RBCs has possibly caused some damage to his liver, so a liver biopsy is being discussed to determine the iron level and if there is any damage to the liver.

There is a very good scientific discussion that includes some information about this genetic combination at http://findarticles.com/p/articles/mi_qa3867/is_200210/ai_n9087640   What is very interesting is that the combination of this triple alpha gene with beta major actually decreases the severity of the thal, but when that alpha is combined with only one beta gene, as with Vincenzo, it worsens the condition.

Quote
The number of functional a genes present determines the clinical picture of the disease. The co-existence of alpha-thalassaemia (-alpha/(alpha)(alpha)) with two severe beta-thalassaemia determinants reduces the severity of beta thalassaemia major. However, coexistence of triplicated alpha-gene with beta-thalassaemia trait increases the severity of this condition.

Vincenzo, I will do my best to explain your posts to our members. Your case is very unusual and very educational. As I said before, a low iron diet and drinking tea with meals, especially iron rich meals is highly recommended.
Andy

All we are saying is give thals a chance.

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Offline jzd24

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Re: Hello to all
« Reply #10 on: November 28, 2006, 06:11:00 AM »
Hi Vincenzo,
     Welcome to the site. We are happy to hear from you.  :hithere   Jean

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Offline C3nZ0

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Re: Hello to all
« Reply #11 on: November 28, 2006, 08:52:13 AM »
Perfect  Andy :biggrin

It's explained very well and complite  :wink

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Offline Manal

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Re: Hello to all
« Reply #12 on: November 28, 2006, 11:38:30 AM »
Hello Andy

Thanks for the explaination. It is now very clear.

The only thing , i don't get is that
This combination is causing chronic hemolysis, which is the premature destruction of red blood cells, in Vincenzo.

Vincenzo's HB level does not show this hemolysis. As he said, his low is 12, but he is usually 14.
HB is supposed the first indicator of hemolysis, isn't it??

Manal

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Offline C3nZ0

  • I'm thal Int, but minor trait
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  • 22
  • Gender: Male
Re: Hello to all
« Reply #13 on: November 28, 2006, 10:41:16 PM »
Vincenzo's HB level does not show this hemolysis. As he said, his low is 12, but he is usually 14.
HB is supposed the first indicator of hemolysis, isn't it??

Sorry is my bad eng. :(
My Hb is usually 12.
 14 is the minimun value considered normal, but I'm always under that
Another value for emolisys is my LDH that is very hight (>700)

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Offline Manal

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Re: Hello to all
« Reply #14 on: November 29, 2006, 12:25:10 AM »
Hi Vincenzo

I guess HB 12  is quiet good even if it is under the normal range. I mean it won't affect growth, bone deformation,......etc
Do we consider having HB 12 a case that suffers hemolysis??

Andy could you please shed some light on this issue

Manal

 

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