What is the pathophysiology of thrombotic thrombocytopenic purpura?
Thrombotic thrombocytopenic purpura (TTP) is a disorder with characteristic von Willebrand factor (VWF)-rich microthrombi affecting the arterioles and capillaries of multiple organs. The disorder frequently leads to early death unless the patients are treated with plasma exchange or infusion.
Which syndrome is caused by the deficiency of plasma enzyme ADAMTS13?
Congenital or acquired deficiency of ADAMTS13 can result in thrombotic thrombocytopenic purpura (see Chapter 134).
Can hypertension cause thrombocytopenia?
Abstract. Patients with malignant hypertension sometimes exhibit microangiopathic hemolytic anemia/thrombocytopenia known as thrombotic microangiopathy (TMA). On the other hand, severe hypertension is sometimes associated with hemolytic uremic syndrome (HUS)/thrombotic thrombocytopenic purpura (TTP).
Is hemolytic anemia associated with hypertensive crisis?
It is established that Focal Segmental Glomerulosclerosis (FSGS) can lead to difficult to control hypertension. It has also been shown that malignant hypertension can cause microangiopathic hemolytic anemia (MAHA).
What is the function of ADAMTS13 enzyme?
The ADAMTS13 enzyme cuts von Willebrand factor into smaller pieces to regulate its interaction with platelets. By processing von Willebrand factor in this way, the enzyme prevents it from triggering the formation of blood clots in normal circulation.
What are the differences between thrombotic thrombocytopenic purpura TTP and haemolytic uremic syndrome HUS )?
HUS is characterized by thrombocytopenia, anaemia and renal insufficiency, whereas the pentad of signs and symptoms including thrombocytopenia, anaemia, neurologic deficit, renal dysfunction and fever is observed in TTP.
How does ADAMTS13 cause TTP?
The deficiency of ADAMTS13 leaves unchecked the hyperadhesive vWF unfolded under high shear stress in the microvessels, resulting in the formation of platelet thrombi, which in turn causes TTP.
What is the important function of ADAMTS13?
Normal Function The ADAMTS13 gene provides instructions for making an enzyme that is involved in regulating blood clotting. After an injury, clots normally protect the body by sealing off damaged blood vessels and preventing further blood loss. The ADAMTS13 enzyme processes a large protein called von Willebrand factor.
What is the most common cause of malignant hypertension?
What causes malignant hypertension? Uncontrolled high blood pressure is one of the main causes of malignant hypertension. Other causes include: Adrenal disorders including Conn’s syndrome, Cushing’s syndrome, pheochromocytoma or a renin-secreting tumor.
How does malignant hypertension cause MAHA?
Microangiopathic hemolysis (MAHA) related to malignant hypertension may cause renal insufficiency by obstruction of interlobular arteries. We hypothesized that the presence of MAHA is an important indicator of renal dysfunction and recovery in malignant hypertension.
What is the most common cause of hypertensive crisis?
What are the causes of hypertensive crisis? The most common cause of hypertensive emergency is an abrupt increase in blood pressure in patients with chronic hypertension. Medication noncompliance is a frequent cause of such changes.
What triggers hypertensive crisis?
The following factors are commonly associated with hypertensive crisis: not taking, or forgetting to take, prescribed blood pressure medications. taking medications that interact with each other in a way that increases blood pressure. using illegal drugs, such as cocaine or amphetamines.