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Thrombotic thrombocytopenic purpura (TTP or Moschcowitz syndrome is a rare disorder of the blood-coagulationsystem, causing extensive microscopic clots to form in the small blood vessels throughout the body. These small blood clots, called thrombi, can harm many organs including the kidneys, heart and brain. In the era before effective treatment with plasma exchange, the death rate was about 90%. With plasma exchange, this has dropped to 10% at six months. Immunosuppressants,suchas glucocorticoids, rituximab, cyclophosphamide, vincristine, or cyclosporine, may also be used if a relapse or recurrence follows plasma interchange. Most cases of TTP arise from obstruction of the enzyme ADAMTS13, a metalloprotease responsible for cleaving large multimers of von Willebrand factor (vWF) into smaller units. The increase in circulating multimers of vWF increase platelet adhesion to areas of endothelial injury, particularly at arteriole-capillary junctions.
A rarer form of TTP called Upshaw-Schülman syndrome, is genetically inherited as a dysfunction of ADAMTS13. If large vWF multimers continues, a tendency for increased coagulation exists. Red blood cells passing the microscopic clots are subjected to shear stress which damages their membranes, leading to intravascularhemolysis, which in turn leads to anaemia and schistocyte formation. Reduced blood flow due to thrombosis and cellular injury results in end organ damage. Current therapy is based on support and plasmapheresis to reduce circulating antibodies against ADAMTS13 and replenish blood levels of the enzyme. TTP is characterized by thrombotic microangiopathy (TMA), the formation of blood clots in small blood vessels throughout the body, which can lead to microangiopathic hemolytic anemia and thrombocytopenia. This characteristic is shared by two related syndromes, hemolytic-uremic syndrome (HUS) and atypical hemolytic-uremic syndrome (aHUS).Consequently, differential diagnosis of these TMA-causing diseases is essential. In addition to TMA, one or more of the following symptoms may be present in each of these diseases: neurological symptoms (e.g. confusion, cerebral convulsions seizures renal impairment(e.g. elevated creatinine, decreased estimated glomerular filtration rate [eGFR], abnormal urinalysisand gastrointestinal (GI) symptoms (e.g. diarrhea nausea/vomiting, abdominal pain, gastroenteritis. Unlike HUS and aHUS, TTP is known to be caused by an acquired defect in the ADAMTS13 protein, so a lab test showing ≤5% of normal ADAMTS13 levels is indicative of TTP. ADAMTS13 levels above 5%, coupled with a positive test for shiga-toxin/enterohemorrhagic E. coli (EHEC), are more likely indicative of HUS, whereas absence of shiga-toxin/EHEC can confirm a diagnosis of aHUS. The symptoms of TTP may at first be subtle, starting with malaise, fever, headache, and sometimes diarrhea. As the condition progresses, clots (thrombi) form within blood vessels, and platelets (clotting cells) are consumed. Bruising, and rarely bleeding, results and may be spontaneous. The bruising often takes the form of purpura, while the most common site of bleeding, if it occurs, is from the nose or gums. Larger bruises (ecchymoses) may also develop.
Thirty four conferences, twenty five National Symposiums and sixteen workshops are organized on thrombotic thrombocytopenic purpura. Three regions in north America Acquired by Thrombotic thrombocytopenic purpura,in central America three regions,in Caribbean one and in south America six regions. In Northern Europe four regions are acquired with thrombotic thrombocytopenic purpura,nine regions in western Europe,nine in central Europe,Eastern Europe six regions,southwestern Europe four,southern Europe two,southeastern Europe seven. One region is acquired with thrombotic thrombocytopenic purpura,central asia three,Eastern Asia seven,south western asia one,southern Asia six,southeastern asia eight. Thrombotic thrombocytopenic purpura, acquired in Western Africa in three regions, in Western Africa seven regions, Central Africa four, Eastern Africa five,south Africa Six.
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Conferences:
• International Conference Thrombotic thrombocytopenic purpura: Disease pathogenesis, laboratory monitoring and current treatment strategies
• Thrombotic Microangiopathy in Malignant Hypertension and Hemolytic Uremic Syndrome (HUS)/Thrombotic Thrombocytopenic Purpura, Department of Nephrology and Endocrinology.
• World congress on controversies in thrombosis and Hemostasis.
• Bari international conference.
• 6th international symposium on women’s health issues in thrombosi and haemostasis.
• Ablynx to present at JP Morgan conference.
• TTP Foundation at the Emergency Medicine Update Conference.
Associations:
• (Thrombotic Thrombocytopenic Purpura) Foundation22 Prince George Dr, Genetic and Rare Diseases (GARD) Information Cente.
• NIH/National Heart.
• Lung and Blood Institute.
• European Hematology Association.
• The International Society on Thrombosis and Haemostasis.
• American Society for Apheresis.
• American Society of Hematology-Self Assessment Program.
• Canadian Society of Nephrology.
• African Society for Immuno deficiencies.
Companies:
• Greenville Hospital System.
• Great Ormond Street Hospital (GOSH).
• UNC Hospitals.
• Christian Medical College and Hospital.
• Sandwell General Hospital.
• Sandwell and West Birmingham Hospitals.
• Baylor College of Medicine and Texas Children's Hospital.
• K.E.M. Hospital.
This page will be updated regularly.
This page was last updated on November 5, 2024
