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Thrombotic Thrombocytopenic Purpura(TTP)

Thrombotic Thrombocytopenic Purpura(TTP)

Last Reviewed : 12/28/2020
Thrombotic Thrombocytopenic Purpura(TTP)

Thrombotic thrombocytopenic purpura (TTP or Moschcowitz syndrome) is a rare disorder of the blood-coagulation system, causing extensive microscopic clots to form in the small blood vessels throughout the body. These small blood clots, called thrombi, can damage many organs including the kidneys, heart and brain. In the era before effective treatment with plasma exchange, the fatality rate was about 90%. With plasma exchange, this has dropped to 10% at six months. Immunosuppressants, such asglucocorticoids, rituximab, cyclophosphamide, vincristine, or ciclosporin, may also be used if a relapse or recurrence follows plasma exchange.

Most cases of TTP arise from autoantibody-mediated inhibition 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 increases platelet adhesion to areas of endothelial injury, particularly at arteriole-capillary junctions.

A rarer form of TTP, called Upshaw–Schulman syndrome, is genetically inherited as a dysfunction of ADAMTS13, resulting in persisting large vWF multimers.

Red blood cells passing the microscopic clots are subjected to shear stress which damages their membranes, leading to rupture of red blood cells within blood vessels, 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 andplasmapheresis to reduce circulating antibodies against ADAMTS13 and replenish blood levels of the enzyme.

We researched this topic for you and found the following best online resources. They are categorized into basic, advanced, and research level based on the extent of information you need. You will be taken to the respective websites by pressing on the links below.

 

 

Basic information:

https://www.nlm.nih.gov/medlineplus/ency/article/000552.htm medline plus thrombotic thrombocytopenic purpura

https://www.nhlbi.nih.gov/health/health-topics/topics/ttp national heart lung and blood institute what is thrombotic thrombocytopenic purpura?

http://www.merckmanuals.com/professional/hematology-and-oncology/thrombocytopenia-and-platelet-dysfunction/thrombotic-thrombocytopenic-purpura-ttp-and-hemolytic-uremic-syndrome-hus merck manuals(professional version) thrombotic thrombocytopenic purpura (ttp) and hemolytic-uremic syndrome (hus)

http://www.healthline.com/health/thrombotic-thrombocytopenic-purpura healthline thrombotic thrombocytopenic purpura

http://www.medicinenet.com/script/main/art.asp?articlekey=13855 medicine net.com definition of thrombotic thrombocytopenic purpura (ttp)

https://www.answeringttp.org/ttp-overview answering ttp thrombotic thrombocytopenic purpura

http://www.netdoctor.co.uk/conditions/heart-and-blood/a1178/thrombotic-thrombocytopenic-purpura-ttp/ net doctor.co thrombotic thrombocytopenic purpura

http://www.webmd.com/a-to-z-guides/thrombotic-thrombocytopenic-purpura-10665 web md thrombotic thrombocytopenic purpura

http://lifeinthefastlane.com/ccc/thrombocytopaenic-thrombotic-purpura-ttp/ life in the fast lane thrombotic thrombocytopenic purpura

http://www.nytimes.com/health/guides/disease/thrombotic-thrombocytopenic-purpura/overview.html newyork times thrombotic thrombocytopenic purpura

http://www.mayoclinic.org/diseases-conditions/idiopathic-thrombocytopenic-purpura/home/ovc-20201208 mayoclinic thrombotic thrombocytopenic purpura

https://www.verywell.com/thrombotic-thrombocytopenic-purpura-401305 very well thrombotic thrombocytopenic purpura

 

Advanced information:

http://emedicine.medscape.com/article/206598-overview medscape thrombotic thrombocytopenic purpura (ttp)

http://www.bcshguidelines.com/documents/bjh_ttp_guideline_0512.pdf guidelines on the diagnosis and management of thrombocytopenic purpura and other thrombotic microangiopathies by marie scull et al., british journal of haematology doi:10.1111/j.1365-2141.2012.09167.x

http://patient.info/in/doctor/thrombotic-thrombocytopenic-purpura-pro patient thrombotic thrombocytopenic purpura

http://rarediseases.org/rare-diseases/thrombotic-thrombocytopenic-purpura/ national organisation of rare diseases thrombotic thrombocytopenic purpura

http://www.fpnotebook.com/hemeonc/platelet/thrmbtcthrmbcytpncprpr.htm family practise notebook thrombotic thrombocytopenic purpura

http://www.medbullets.com/step1-heme/7031/thrombotic-thrombocytopenic-purpura-ttp med bullets thrombotic thrombocytopenic purpura

http://www.healio.com/hematology-oncology/news/print/hemonc-today/%7b1dfede30-982e-463e-96b2-8b83fbd01521%7d/update-on-thrombotic-thrombocytopenic-purpura healio update on thrombotic thrombocytopenic purpura

http://www.dovemed.com/diseases-conditions/thrombotic-thrombocytopenic-purpura/ dove med thrombotic thrombocytopenic purpura

 

Research:

http://www.hindawi.com/journals/crim/2011/953890/ case reports in medicine volume 2011 (2011), article id 953890, 5 pages
http://dx.doi.org/10.1155/2011/953890 case report:thrombotic thrombocytopenic purpura associated with mixed connective tissue disease: a case report by joão tadeu damian souto filho et al.,

niaz fa, aleem a. response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus. saudi j kidney dis transpl [serial online] 2010 [cited 2016 jun 19];21:109-12. available from: http://www.sjkdt.org/text.asp?2010/21/1/109/58783

http://www.sciencedirect.com/science/article/pii/s2352396415300396 pathogenicity of anti-adamts13 autoantibodies in acquired thrombotic thrombocytopenic purpura by mari r. thomas et al., ebiomedicine volume 2, issue 8, august 2015, pages 942–952 doi:10.1016/j.ebiom.2015.06.007

de serres sa, isenring p. renal thrombotic microangiopathy revisited: when a lesion is not a clinical finding. saudi j kidney dis transpl [serial online] 2010 [cited 2016 jun 19];21:411-6. available from: http://www.sjkdt.org/text.asp?2010/21/3/411/62689

http://europepmc.org/backend/ptpmcrender.fcgi?accid=pmc3299727&blobtype=pdf matsumoto m, bennett cl, isonishi a, qureshi z, hori y, et al. (2012) acquired idiopathic adamts13 activity deficient thrombotic thrombocytopenic purpura in a population from japan. plos one 7(3): e33029. doi:10.1371/journal.pone.0033029

http://www.scopemed.org/fulltextpdf.php?mno=174626 staphylococcus aureus septicemia presenting as disseminated intravascular coagulation - thrombotic thrombocytopenic purpura overlap and thrombus in inferior vena cava, right atrium and right ventricle: a case report by khwaja saifullah zafar, p. s. singh, pulak raj international journal of research in medical sciences zafar ks et al. int j res med sci. 2015 jan;3(1):368-372 doi: 10.5455/2320-6012.ijrms20150175

http://www.hindawi.com/journals/crionm/2012/619348/ case reports in oncological medicine
volume 2012 (2012), article id 619348, 5 pages http://dx.doi.org/10.1155/2012/619348 case report:recurrent thrombotic thrombocytopenic purpura-like syndrome as a paraneoplastic phenomenon in malignant peritoneal mesothelioma: a case report and review of the literature by francisco socola et al.,

parikh p m, chile v s, mehta b c, anjaria p d. thrombotic thrombocytopenic purpura (a case report). j postgrad med [serial online] 1987 [cited 2016 jun 19];33:105. available from: http://www.jpgmonline.com/text.asp?1987/33/2/105/5296

nasa p, dua j m, kansal s, chadha g, chawla r, manchanda m. life-threatening postpartum hemolysis, elevated liver functions tests, low platelets syndrome versus thrombocytopenic purpura - therapeutic plasma exchange is the answer. indian j crit care med [serial online] 2011 [cited 2016 jun 19];15:126-9. available from: http://www.ijccm.org/text.asp?2011/15/2/126/83016

hawley j s, ney jp, swanberg m. thrombotic thrombocytopenic purpura -induced posterior leukoencephalopathy in a patient without significant renal or hypertensive complications . j postgrad med [serial online] 2004 [cited 2016 jun 19];50:197-9. available from: http://www.jpgmonline.com/text.asp?2004/50/3/197/12573

 

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