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Let’s talk about thrombotic thrombocytopenic purpura or TTP. It is one of those hematologic conditions which we don’t see much of but when we suspect it, its a real life-threatening emergency. It may also seeem like a nebulous concept at first but my goal here is to simplify it and focus on the practical aspects.
Simply put- TTP is a clinical condition in which there is widespread microthrombosis in the body. The microthrombi can be present anywhere but most commonly, it presents in the brain with strokes on imaging.
What exactly causes TTP?
TTP is precipitated when the body doesn’t have enough of a protein called ADAMTS13. Low ADAMTS13 can be due to congenital deficiency (which is rare, thank goodness!) or an acquired antibody that inhibits the ADAMTS13 suppressing its activity.
The full form of ADAMTS13 is A Disintegrin And Metalloproteinase with a ThromboSpondin type 1 motif, member 13- its a mouthful, I know!
ADAMTS13 normally cleaves the large von Willebrand factor multimers into smaller more manageable ones but when its either inhibited or deficient, this leads to unchecked activity of vWF multimers and widespread platelet aggregation.
Credit- Medbullets
The presentation of TTP, while sounding dramatic, is typically more insidious. Patients present with symptoms lasting a few days to a couple of weeks. High index of suspicion is key in identifying and managing TTP.
As the microthrombi start causing blood vessels to occlude, they also lead to shearing of the red blood cells causing them to break apart into fragments called schistocytes which is a hallmark of TTP
Think TTP when you see low hemoglobin (anemia) and low platelets (thrombocytopenia) together. The platelet count tends to be <50,000/microliter
In the next edition, I will talk about the initial management of TTP. Stay tuned!
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Disclaimer: The content in this newsletter is for educational purposes only and should not be considered medical advice. Subscription to this newsletter does not establish a doctor-patient relationship. Always consult your physician with specific questions regarding your health
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