Danger Level: High
What is it?
TTP is a life threatening disease in which the platelets (the cells in the blood responsible for blood clotting) are missing and the red blood cells are destroyed.
Who gets it?
It’s a rare disease: 1 person out of 4 million a year will have the disease. It usually affects men more than women (but not by much), and it usually happens in the ages of 30-40.
What causes it?
When a blood clot is formed, those platelets mentioned above aggregate together to seal the wound out of which blood is coming out. They do this with the help of many small molecules, one of them is VWF – or by its long name: Von-Willebrand Factor.
In TTP there is a problem with VWF – it’s getting larger and larger because the mechanism responsible for cutting it to its appropriate size is defective. That’s why platelets are aggregating like crazy and block blood vessels. This causes the destruction of normal blood cells and the other things mentioned below.
How does it feel?
5 things happen to you if you get TPP:
- Confusion, seizure and even coma: Because those platelets that block blood vessels won’t allow blood to go to your brain properly.
- Renal failure: Your kidneys will stop working, because blood won’t go to your kidneys as well.
- Fever
- The number of platelets goes down: Because they’re all clumped together
- Anemia: Which is loss of your red blood cells. It happens because they are destroyed when bumping into those clumps.
How is it discovered?
Whenever someone comes to the ER with those 5 things (or some of them), the doctor will quickly take some blood out and do 2 things:
- Look under the microscope: Where they will see those destroyed blood cells. They will look something like the picture below.
- Let the lab count it: The count will show that the number of platelets is down, and things that go up whenever blood is destroyed will go up.
You can see that some of the cells are round, but some are deformed. (Photo by Ed Uthman, MD, Some Rights Reserved)
How is it treated?
The best way to answer that: FAST! Without the proper treatment, there is about 100% chance of dying from TTP.
The treatment involves a process called plasmapharesis, in which blood is removed from the body and blood from a donor is returned back, this way taking out the elements in the blood that caused the disease.
What happens after treatment?
In 3 weeks up to 90% of people will be mostly disease free. But: The disease can come back in 1/3 of people in the months and years that follow. They should get their blood checked periodically!
The Bottom Line: How do I avoid it?
Unfortunately, there is really nothing you can do to avoid it. If you fear that you have TTP, according to the information above, you should go to the emergency department immediately.
What next?
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