Thrombotic Thrombocytopenic Purpura (TTP)
A CBC is one of the most common ED lab studies obtained, and while this “basic lab” is something that is often ordered reflexively, perhaps to check for anemia or leukocytosis, the sometimes-forgotten third major component of this test is the platelet level - Sarah Moulds MD
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What Is TTP?
While there are no specific symptoms for TTP, the majority (>70%) of patients present with neurological symptoms, which can range from headache and visual disturbance to more severe symptoms, including TIA or CVA. The haemolysis and resulting anaemia can cause progressive fatigue and SOB, as well as dark urine/haematuria. While bruising symptoms can occur, bleeding symptoms are not usually seen, despite severe thrombocytopenia. Cardiac involvement is not usually associated with symptoms other than in particularly severe cases, but usually detected via the finding of an elevated Troponin.
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Update on Thrombotic Thrombocytopenic Purpura
Thrombotic thrombocytopenic purpura (TTP) is a microangiopathic disorder, which has become remarkably better understood over the last 3 decades. This disease, with a nearly 90% mortality rate when left untreated, is now managed with great success and typically without long-term sequela
Articles of Interest
About TTP
People with TTP have a deficiency of the enzyme that should break down the von Willebrand Factor, which, with platelets, normally prevents bleeding. This enzyme stops working properly and the platelets become sticky and form blood clots in small vessels that can affect any organ. Thrombotic Thrombocytopenic Purpura (TTP) is known, in the majority of patients, as an autoimmune condition.
Thrombotic Thrombocytopenic Purpura (also known as ‘TTP’)
Von Willebrand factor (VWF) is an important substance in the blood to help with normal blood clotting. Platelets are a type of cell found in the blood which help to form blood clots. After a cut or bruise, platelets and VWF work together to seal the injury forming a blood clot. Usually an enzyme called ADAMTS13 breaks down large VWF in the blood and regulates its levels. In TTP there is an excess of large VWF which should not be present. This large VWF causes platelets to get stuck in the small blood vessels throughout the body.
Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder
There are 2 types of TTP, acquired and hereditary. Acquired TTP is caused by a problem in your immune system. Hereditary TTP is caused by a problem in a gene passed down through your family. Both types of TTP lead to not having enough of a protein called ADAMTS13 in your blood. ADAMTS13 helps your blood flow normally and clot only when your body needs it.
Thrombotic Thrombocytopenic Purpura Evaluation and Management
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening condition. Most cases are related to a severe deficiency of ADAMTS13 enzyme in the blood. TTP is considered a hematologic emergency because, even with treatment, the mortality is estimated at 10% to 20%. The diagnosis is challenging given the significant overlap of the clinical presentation of TTP and other conditions, especially other thrombotic microangiopathies.
Resources
National TTP Group
The National TTP Group represents the nine specialist centres that treat Thrombotic Thrombocytopenic Purpura (TTP) in England. We are commissioned by NHS England to treat all cases of TTP and provide inpatient care for TTP, as well as long-term monitoring to prevent TTP relapse.
TTP Network
A patient led organisation supporting those diagnosed with Thrombotic Thrombocytopenic Purpura (TTP), and their families. Our mission is to improve outcomes and quality of life for patients and families living with TTP, through support, education and advocacy ensuring that no one facing TTP will feel alone.
Understanding TTP
When recovering from TTP, some people can feel eager to return to their normal lives. Everyone has a different experience of TTP, so everyone has a different recovery. Your recovery should be guided by how you feel, and it should happen on your own timeline. Take things slowly and listen to your body.
NORD
The exact cause of TTP is not known. However, the disease is associated with a deficiency of an enzyme involved in blood clotting called the von Willebrand factor cleaving protease (also called ADAMTS13). The deficiency of this enzyme allows large complexes of the clotting protein known as von Willebrand factor to circulate in the blood, resulting in platelet clotting and the destruction of red blood cells.
