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. Download Citation on ResearchGate | Púrpura trombocitopénica trombótica puerperal | Thrombotic thrombocytopenic purpura is a multisystem disease of. Download Citation on ResearchGate | Púrpura trombocitopénica trombótica refractaria | Thrombotic thrombocytopenic purpura (TTP) is a rare immune.
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Acute morbidities include ischemic events such as stroke, transient ischemic attacks, myocardial infarction and cardiac arrhythmia, bleeding, and azotemia. New England Journal of Medicine. Related Bing Images Extra: A closely related disorder, hemolytic-uremic syndrome HUSshares many clinical characteristics of TTP but is more common in children. See Treatment and Medication for more detail. Send the link below via email or IM.
Check out this article to learn more or contact your system administrator. Platelets are consumed in the aggregation teombocitopenica and bind vWF.
Large bruisesfeverweakness, shortness of breathconfusion, headache  . This is fresh frozen plasma that has had the cryoprecipitate removed and is thus depleted of high-molecular-weight von Willebrand multimers, which have a pathogenic role in TTP.
Bouw et al have presented a review article of TTP in children. It is an acute or subacute condition. Rituximab, although not trombootica for use in TTP, trombotifa increasingly recommended for use in refractory cases. About 1 perpeople are affected. Exact incidence figures for the United States are not available, although TTP is thought to be a rare disease. These thrombi consist predominantly of platelets with little fibrin and red cells compared with thrombi that occur secondary to intravascular coagulation.
Although the two disorders were once considered variants of a single syndrome, current evidence suggests differing pathogenic trommbotica of TTP and HUS. Coagulopathies Autoimmune diseases Rare diseases Vascular-related cutaneous conditions Syndromes affecting blood. In one series, the frequency was approximately 1 in 50, hospital admissions.
The signs and symptoms of TTP may at first be subtle and nonspecific. Epidermal wart callus seborrheic keratosis acrochordon molluscum contagiosum actinic keratosis squamous-cell carcinoma basal-cell carcinoma Merkel-cell carcinoma nevus sebaceous trichoepithelioma.
Very large vWF multimers are more prone to lead to coagulation. While a response to blood transfusion had been noted before, a report and subsequent studies showed blood plasma was highly effective in improving the disease process.
Houston, we have a problem! Proc NY Pathol Soc. Platelet transfusions and bleeding purpira associated with plasma exchange catheter placement in patients with presumed thrombotic thrombocytopenic purpura.
TTP during pregnancy may precipitate fetal loss. Due to the high mortality of untreated TTP, a presumptive diagnosis of Trombocitipenica is made even when only microangiopathic hemolytic anemia and thrombocytopenia are seen, and therapy is started.
Patients with TTP typically report an acute or subacute onset of symptoms related to neurologic dysfunction, anemia, or thrombocytopenia. Please log in to add your comment. Back Links pages that link to this page. Severe bleeding from thrombocytopenia is unusual, although petechiae are common.
Search Tronbocitopenica for all related images. Caplacizumab for Acquired Thrombotic Thrombocytopenic Purpura.
Share Email Print Feedback Close. Thrombotic thrombocytopenic purpura and pregnancy: Neither you, nor the coeditors you shared it with will be able to recover it again. The syndrome described by Moschowitz is now known as thrombotic thrombocytopenic purpura TTP. Hypo- coagulability Thrombocytopenia Thrombocytopenic purpura: Secondary TTP has been associated with the use of certain drugs, including chemotherapy drugs such as gemcitabine and mitomycin and antiplatelet agents such as clopidogrel and ticlopidine.
Content is updated monthly with systematic literature reviews and conferences.
Practice Essentials Thrombotic thrombocytopenic purpura TTP is a rare blood disorder characterized by clotting in small blood vessels thrombosesresulting in a low platelet count.
In the congenital form of TTP, mutations in the gene encoding this protease have been described. In those patients refractory to plasma exchange, using cryopoor plasma or cryosupernatant has sometimes led to a response.
Lippincott, Williams, and Wilkins.
Probable etiology may involve, at least in some cases, endothelial damage,  although the formation of thrombi resulting in vessel occlusion may not be essential in the pathogenesis of secondary TTP. Annual Review of Medicine.
Since trombocitopencia early s, plasmapheresis has become the treatment of choice for TTP. As platelets are used up in the formation of trokbocitopenica, this then leads to a decrease in the number of overall circulating platelets, which may then cause life-threatening bleeds. The underlying mechanism typically involves autoantibody-mediated inhibition of the enzyme ADAMTS13a metalloprotease responsible for cleaving large multimers of von Willebrand factor vWF into smaller units.
Do you really want to delete this prezi? Frequently reported symptoms include feeling very tiredconfusionand headaches. Some tolerate longer intervals between plasma infusions.
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