Thrombotic thrombocytopenic purpura (TTP) is a blood disorder that results in blood clots forming in small blood vessels throughout the body.[2] This results in a low platelet count, low red blood cells due to their breakdown, and often kidney, heart, and brain dysfunction.[1] Symptoms may include large bruises, fever, weakness, shortness of breath, confusion, and headache.[2][3] Repeated episodes may occur.[3]
In about half of cases a trigger is identified, while in the remainder the cause remains unknown.[3] Known triggers include bacterial infections, certain medications, autoimmune diseases such as lupus, and pregnancy.[3] The underlying mechanism typically involves antibodies inhibiting the enzyme ADAMTS13.[1] This results in decreased break down of large multimers of von Willebrand factor (vWF) into smaller units.[1] Less commonly TTP is inherited, known as Upshaw–Schulman syndrome, such that ADAMTS13 dysfunction is present from birth.[5] Diagnosis is typically based on symptoms and blood tests.[2] It may be supported by measuring activity of or antibodies against ADAMTS13.[2]
With plasma exchange the risk of death has decreased from more than 90% to less than 20%.[1] Immunosuppressants, such as glucocorticoids, and rituximab may also be used.[3] Platelet transfusions are generally not recommended.[6]
About 1 per 100,000 people are affected.[3] Onset is typically in adulthood and women are more often affected.[3] About 10% of cases begin in childhood.[3] The condition was first described by Eli Moschcowitz in 1924.[3] The underlying mechanism was determined in the 1980s and 1990s.[3]
^ abcdefg"Immune-mediated thrombotic thrombocytopenic purpura". Genetic and Rare Diseases Information Center. U.S. Department of Health & Human Services. Archived from the original on 2018-10-20. Retrieved 2018-10-10.
Thromboticthrombocytopenicpurpura (TTP) is a blood disorder that results in blood clots forming in small blood vessels throughout the body. This results...
to be immune-mediated.[citation needed] Another form is thromboticthrombocytopenicpurpura.[citation needed] Diagnosis is done by the help of symptoms...
thrombocytopenicpurpura (ITP), also known as idiopathic thrombocytopenicpurpura or immune thrombocytopenia, is a type of thrombocytopenicpurpura characterized...
Thromboticthrombocytopenicpurpura (TTP) is a life-threatening disorder characterized by thrombocytopenia and microangiopathic hemolytic anemia accompanied...
artificial heart valves, hemolytic uremic syndrome, and thromboticthrombocytopenicpurpura, among other causes. Excessive schistocytes present in blood...
thrombocytopenia, anemia, purpura and kidney failure. The classic TMAs are hemolytic uremic syndrome and thromboticthrombocytopenicpurpura. Other conditions...
Purpura fulminans is an acute, often fatal, thrombotic disorder which manifests as blood spots, bruising and discolouration of the skin resulting from...
disease and is involved in many other diseases, including thromboticthrombocytopenicpurpura, Heyde's syndrome, and possibly hemolytic–uremic syndrome...
heartburn. More severe side effects include bleeding and thromboticthrombocytopenicpurpura. While there is no evidence of harm from use during pregnancy...
Guillain–Barré syndrome, lupus, myasthenia gravis, and thromboticthrombocytopenicpurpura. During plasmapheresis, blood, which consists of blood cells...
bites and known to cause sepsis, potentially complicated by thromboticthrombocytopenicpurpura and hemolytic uremic syndrome, in immunocompromised patients...
uremic syndrome, disseminated intravascular coagulation, thromboticthrombocytopenicpurpura, and malignant hypertension, the endothelial layer of small...
single-domain antibody (VHH) designed for the treatment of thromboticthrombocytopenicpurpura (TTP) and thrombosis. This drug was developed by Ablynx NV...
surviving family and friends revealed complications from thromboticthrombocytopenicpurpura as his cause of death. He is buried at St. Anthony's & St...
IVIG) has largely replaced fresh frozen plasma Treatment of thromboticthrombocytopenicpurpura: Therapeutic plasma exchange with FFP/PF24 or thawed plasma...
occasions, been suggested to cause hemolytic uremic syndrome and thromboticthrombocytopenicpurpura, though no unequivocal case reports exist. Campylobacter...
with other conditions, such as acute kidney injury and thromboticthrombocytopenicpurpura. Increasing the understanding of HELLP syndrome's pathophysiology...
ninth chromosome (9q34). Since 1982 it had been known that thromboticthrombocytopenicpurpura (TTP), one of the microangiopathic hemolytic anemias (see...
PMID 28428120. Sadler, J. Evan (2017-09-07). "Pathophysiology of thromboticthrombocytopenicpurpura". Blood. 130 (10). American Society of Hematology: 1181–1188...
resembles a combination of autoimmune hemolytic anemia and immune thrombocytopenicpurpura. Autoimmune hemolytic anemia is a condition in which the red blood...
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