Half of the cases doesn’t know the cause for the disease. Is a life treating disease
the general symptoms for TTP are; fever, distress, acute renal insufficiency, anemia, weakness, chronic fatigue, confusion, bleeding, and brushing.
Plasmapheresis- blood is removed from the body and blood from a donor is returned backImmunosuppressive therapies- the reduction in the effectiveness of the immune system.
VWF multimers also form a bridge between the platelets themselves as a mediator of platelet aggregation. Once the vascular injury is repaired (right), formation of the platelet plug is downregulated thanks to ADAMTS13, which specifically cleaves the large VWF multimers in smaller forms that are less adhesive to plateletshttp://www.youtube.com/watch?v=0pnpoEy0eYE
What is ThromboticThrombocytopenic Purpura (TTP)? Severe and rareblood disorder Affects thecoagulation ofdiverse organs ofthe body,specifically minorveins Women are themost affected 4-7 million peopleare diagnosedeach year in theUnited Stateshttp://www.google.com.pr/imgres?q=thrombotic+thrombocytopenic+purpura+pictures&um=1&hl=es&biw=1366&bih=646&tbm=isch&tbnid=NbLkpWPDNlWFgM:&imgrefurl=http://www.health-pic.com/thrombotic-thrombocytopenic-purpura-ppt/&docid=hQXqEmiueSJ_mM&imgurl=http://www.health-pic.com/EX/09-19-03/29345-0550x0475.jpg&w=475&h=550&ei=r4irULSVLIfm8QTD-oDIBg&zoom=1&iact=hc&vpx=536&vpy=80&dur=30&hovh=242&hovw=209&tx=148&ty=163&sig=105786173697079870779&page=1&tbnh=152&tbnw=131&start=0&ndsp=17&ved=1t:429,r:2,s:0,i:69
Types of TTP Upshaw-Schulman Syndrome –-Hereditary mutation of ADAMTS-13 gene Immune Thrombotic Thrombocytopenic Purpura :-Autoimmune disorder-Low platelet count- Mucocutaneous bleeding Secondary Thrombotic ThrombocytopenicPurpura:- Complication of primary disease orcondition
Treatment No current treatment for the disease. Plasma exchange is usually used Adults:- Plasmapheresis and immunosuppressivetherapies Childs:- Plasma exchange associated with steroids andthe use of rituximab (Bredlau 2011).
Objective Determine the main cause for the three types ofTTP Which one occurs more frequently?MainCause?Upshaw-SchulmanSyndromeImmuneSecondary
Upshaw-Schulman Syndrome TTP is inherited “…due to mutations in the genes encodingcomplement factor H, factor I, factor B ormembrane co factor protein” (Loirat et al. 2009) Has more established factors of diagnostic andtreatment. Studies are trying to find the complications whenanother disease is involved.
Secondary ThromboticThrombocytopenia Purpura (STP) Occurs as a complication of a primary disease- Certain cancers, pregnancy, and HIV Pregnancy is the most common cause for STP Measurement of ADAMTS-13 activity, ADAMTS-13 inhibitor, ADAMTS-13 antigen, and ADAMTS-13 gene analysis is primordial to understand therole of ADAMTS-13 in USS, during pregnancy.
Immune ThromboticThrombocytopenic Purpura (ITP) Autoimmune disorder, characterized by a lowplatelet count and mucocutaneous bleeding. Incidence 100 cases per 1 million persons peryear. There is not a main cause established and it isnot developed as a secondary effect. Scientists are searching for a common factor
Major Conclusion These are all related to the deficient activity of thevon Willebrand factor (VWF) cleaving proteaseADAMTS-13.http://www.tcd.ie/IMM/haemostasis/projects/von-willebrand-factor.php
Minor Conclusions The most common is STP Statistics concerning this topic were not found. Itis essential to have the statistics in order to obtainreliable results. Immune Thrombotic Thrombocytopenia Purpurais of more interest for investigations. Develop better treatments and diagnostics.