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Concept
Thrombotic thrombocytopenic purpura
Summary
Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that results in blood clots forming in small blood vessels throughout the body. This results in a low platelet count, low red blood cells due to their breakdown, and often kidney, heart, and brain dysfunction. Symptoms may include large bruises, fever, weakness, shortness of breath, confusion, and headache. Repeated episodes may occur.
In about half of cases a trigger is identified, while in the remainder the cause remains unknown. Known triggers include bacterial infections, certain medications, autoimmune diseases such as lupus, and pregnancy. The underlying mechanism typically involves antibodies inhibiting the enzyme ADAMTS13. This results in decreased break down of large multimers of von Willebrand factor (vWF) into smaller units. Less commonly TTP is inherited, known as Upshaw–Schulman syndrome, such that ADAMTS13 dysfunction is present from birth. Diagnosis is typically based on symptoms and blood tests. It may be supported by measuring activity of or antibodies against ADAMTS13.
With plasma exchange the risk of death has decreased from more than 90% to less than 20%. Immunosuppressants, such as glucocorticoids, and rituximab may also be used. Platelet transfusions are generally not recommended.
About 1 per 100,000 people are affected. Onset is typically in adulthood and women are more often affected. About 10% of cases begin in childhood. The condition was first described by Eli Moschcowitz in 1924. The underlying mechanism was determined in the 1980s and 1990s.
The signs and symptoms of TTP may at first be subtle and nonspecific. Many people experience an influenza-like or diarrheal illness before developing TTP. Neurological symptoms are very common and vary greatly in severity. Frequently reported symptoms include feeling very tired, confusion, and headaches. Seizures and symptoms similar to those of a stroke can also be seen. Other symptoms include, but are not limited to jaundice or paleness of the skin, a fast heart rate or shortness of breath, or pinpoint-sized purple or reddish dots on the skin known as petechiae.
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Ontological neighbourhood
Related publications (6)
De novo identification of lipid II binding lipopeptides with antibacterial activity against vancomycin-resistant bacteria
Creative strategies for identifying new antibiotics are essential to addressing the looming threat of a post-antibiotic era. We here report the use of a targeted peptide phage display screen as a means of generating novel antimicrobial lipopeptides. Specif ...
Royal Soc Chemistry2017Development of a selective peptide macrocycle inhibitor of coagulation factor XII toward the generation of a safe antithrombotic therapy
Christian Heinis, Philippe Diderich, Vanessa Baeriswyl
Inhibition of coagulation factor XII (FXII) activity represents an attractive approach for the treatment and prevention of thrombotic diseases. The few existing FXII inhibitors suffer from low selectivity. Using phage display combined to rational design, w ...
2013The Angiotensin-Converting Enzyme 2/Angiotensin-(1-7)/Mas receptor axis: A potential target for treating thrombotic diseases
Nikolaos Stergiopulos, Danielle Gomes Passos Silva
Despite many therapeutic advances leading to increasingly effective drug treatments, thrombotic events (such as ischaemic stroke, pulmonary embolism, deep venous thrombosis and acute myocardial infarction) still represent a major worldwide cause of morbidi ...
Schattauer Gmbh-Verlag Medizin Naturwissenschaften2012Related concepts (18)
Hemolytic–uremic syndrome
Hemolytic–uremic syndrome (HUS) is a group of blood disorders characterized by low red blood cells, acute kidney failure, and low platelets. Initial symptoms typically include bloody diarrhea, fever, vomiting, and weakness. Kidney problems and low platelets then occur as the diarrhea progresses. Children are more commonly affected, but most children recover without permanent damage to their health, although some children may have serious and sometimes life-threatening complications.
Disseminated intravascular coagulation
Disseminated intravascular coagulation (DIC) is a condition in which blood clots form throughout the body, blocking small blood vessels. Symptoms may include chest pain, shortness of breath, leg pain, problems speaking, or problems moving parts of the body. As clotting factors and platelets are used up, bleeding may occur. This may include blood in the urine, blood in the stool, or bleeding into the skin. Complications may include organ failure. Relatively common causes include sepsis, surgery, major trauma, cancer, and complications of pregnancy.
Plasmapheresis
Plasmapheresis (from the Greek πλάσμα, plasma, something molded, and ἀφαίρεσις aphairesis, taking away) is the removal, treatment, and return or exchange of blood plasma or components thereof from and to the blood circulation. It is thus an extracorporeal therapy, a medical procedure performed outside the body. Three general types of plasmapheresis can be distinguished: Autologuous, removing blood plasma, treating it in some way, and returning it to the same person, as a therapy.