Re: Essential Thrombocythemia [00041] Medicine On Line


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Re: Essential Thrombocythemia



>My wife has ET.  She is 37 and taking the experimental drug Anagrelide to
>reduce her platelet counts.  I would like to know more about this clonal
>disorder and get a better idea of the prognosis for someone so young.  She
>presented with symptoms and they do return when the platelet levels get very
>high.  Thank you.

Thrombocytopenia is defined as a platelet count below 100,000 per microliter. 
Except for chronic, longstanding thrombocytopenia, hemorrahge is inversely 
proportional to the paltelet count (especially in disorders of platelet 
production). Platelet counts in the range of 40,000-60,000 per microliter may 
lead to post-traumatic bleeding, and at 20,000 per microliter, spontaneous 
hemorrhage can occur. Particularly hazardous are central nervous system and 
gastrointestinal hemorraghe. Fever, anemia, and chronic inflammation in 
thrombocytopenic patients render them more susceptible to bleeding and less 
responsive to platelet transfusions.

There are four basic mechanisms for thrombocytopenia:

1. decreased or ineffective platelet production;
2. shortened platelet survival time in the circulation due to increased
   destruction or consumption or both;
3. splenic sequestration;
4. intravascular dilution of circulating platelets.

This can be investigated to determine which one is responsible...

I'm not familiar with the drug you mentioned in your post. The treatment is 
dependent on the mechanism of the disease...and what has been used to treat it 
so far...

--
E. Loren Buhle, Jr., Ph.D          INTERNET: BUHLEL01@MCRCR6.MED.NYU.EDU
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