TTP is a rare clotting disorder that is caused by a lack of the enzyme ADAMTS13.
Normally, this enzyme cuts the von Willebrand factor into smaller pieces. If there is a shortage of the enzyme, the level of von Willebrand factor in the blood increases which in turn increases the risk of thrombosis. If this condition is left untreated, then there is a substantial risk of the patient dying. Therefore, a quick diagnosis is of vital importance. Making a quick diagnosis has become much easier thanks to the introduction of a one-hour test for ADAMTS13 levels. In her doctoral thesis, Evelyn Groot claims that patients with TTP who have low blood levels of ADAMTS13 during their recovery period are at the greatest risk of a recurrence of the condition. Therefore, it is absolutely essential that these patients are monitored very closely.
The research conducted by Evelyn Groot into the rare condition of TTP also increases understanding the very common condition of malaria. Just like patients with TTP, the symptoms of patients with malaria can be partly explained by a disturbance in the balance between ADAMTS13 and von Willebrand factor. Future therapy is aimed at hindering the interaction between active von Willebrand factor and blood platelets may possibly make a valuable contribution to the recovery of TTP and malaria patients.
Evelyn Groot will receive her PhD on September 24 from Utrecht University. The title of her dissertation is “Active von Willebrand factor in thrombotic thrombocytopenic purpura and malaria.”