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5 Facts you should know about
Congenital thrombotic thrombocytopenic purpura
1
Symptoms generally are due to hemolytic anemia, thrombocytopenia, and neurologic dysfunction
2
The clinical manifestations include the concomitant occurrence of often severe thrombocytopenia, microangiopathic hemolytic anemia, and ischemic organ damage
3
Widespread thrombosis can lead to problems with the nervous system (such as personality changes, headaches, confusion, and seizures), abnormal kidney function, heart problems, and gastrointestinal problems
4
Long-term, there is a risk of end-organ damage, seen in 75% of patients with late diagnosis of cTTP
5
The underlying pathophysiology is an absent or severely reduced activity of ADAMTS13 caused by bi-allelic mutations in the ADAMTS13 gene
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