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Thrombotic thrombocytopenic purpura

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https://www.readbyqxmd.com/read/29444324/neuro-behcet-s-disease-in-a-patient-with-thrombotic-thrombocytopenic-purpura
#1
Stoyan Karaivanov, Anne Krueger, Nicole Bethke, Helena Radbruch, Kai-Uwe Eckardt, Adrian Schreiber
No abstract text is available yet for this article.
February 12, 2018: Rheumatology
https://www.readbyqxmd.com/read/29437728/large-vessel-stroke-as-initial-presentation-of-thrombotic-thrombocytopenic-purpura
#2
Ryan Sugarman, Andrea M Tufano, Johnson M Liu
A 67-year-old right-handed woman presented with dysarthria, left upper extremity weakness and right-sided neglect of 3 hours duration. Imaging of the brain revealed acute right middle cerebral artery stroke; however, tissue plasminogen activator could not be administered due to severe thrombocytopenia. A peripheral smear revealed schistocytes and the patient was treated empirically for thrombotic thrombocytopenic purpura (TTP) with therapeutic plasma exchange. An extensive workup revealed no embolic source or other cause for stroke, and a diagnosis of large vessel infarct secondary to TTP was made...
February 5, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29426622/atypical-hemolytic-uremic-syndrome-an-unusual-postoperative-complication
#3
S Mota, C Filipe, A L Almeida
INTRODUCTION AND OBJECTIVES: Thrombotic thrombocytopenic purpura and atypical haemolytic uremic syndrome (aHUS) are acute, rare, life-threatening thrombotic microangiopathies that require swift management. We report a case of acute microangiopathic haemolytic anaemia (MAHA) presenting in perioperative setting. CLINICAL CASE: After hepatic pericystectomy for hydatid cyst, a 46-year-old female developed MAHA, thrombocytopenia and acute renal failure in the immediate postoperative period...
February 6, 2018: Revista Española de Anestesiología y Reanimación
https://www.readbyqxmd.com/read/29424924/hur-ttp-and-mir-133b-expression-in-nsclc-and-their-association-with-prognosis
#4
L Qian, A-H Ji, W-J Zhang, N Zhao
OBJECTIVE: This study sought to explore HuR, Thrombotic Thrombocytopenic Purpura (TTP), and microRNA 133b (miR-133b) expression levels in non-small cell lung cancer (NSCLC) patients and assess the relationship of expression with disease prognosis. PATIENTS AND METHODS: One hundred and ten paraffin-embedded and 33 fresh flash-frozen NSCLC samples, together with matched tumor adjacent normal tissue controls, were collected from patients between January 2013 and July 2015 in Yidu Central Hospital of Weifang...
January 2018: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/29419916/consensus-opinion-on-diagnosis-and-management-of-thrombotic-microangiopathy-in-australia-and-new-zealand
#5
REVIEW
Lucy Fox, Solomon J Cohney, Joshua Y Kausman, Jake Shortt, Peter D Hughes, Erica M Wood, Nicole M Isbel, Theo de Malmanche, Anne Durkan, Pravin Hissaria, Piers Blombery, Thomas D Barbour
Thrombotic microangiopathy (TMA) arises in a variety of clinical circumstances with the potential to cause significant dysfunction of the kidneys, brain, gastrointestinal tract and heart. TMA should be considered in all patients with thrombocytopenia and anaemia, with an immediate request to the haematology laboratory to look for red cell fragments on a blood film. Whilst TMA of any aetiology generally demands prompt treatment, this is especially so in thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS), where organ failure may be precipitous, irreversible and fatal...
February 8, 2018: Nephrology
https://www.readbyqxmd.com/read/29403592/management-of-retinal-diseases-in-pregnant-patients
#6
REVIEW
Julie M Rosenthal, Mark W Johnson
Pregnancy leads to significant changes in the body, which potentially affect the retina. Pregnancy can induce disease, such as that seen in hypertensive retinopathy and choroidopathy. It can cause exudative retinal detachments in the HELLP syndrome (hemolysis, elevated liver enzymes and low platelets), disseminated intravascular coagulation (DIC), and thrombotic thrombocytopenic purpura (TTP), and provoke arterial and venous retinal occlusive disease. Pregnancy may also exacerbate pre-existing retinal disease, such as idiopathic central serous chorioretinopathy (ICSC) and diabetic retinopathy...
January 2018: Journal of Ophthalmic & Vision Research
https://www.readbyqxmd.com/read/29399327/recent-advances-in-understanding-and-management-of-acquired-thrombocytopenia
#7
REVIEW
Srikanth Nagalla, Ravindra Sarode
There are numerous congenital and acquired causes of thrombocytopenia. Thrombocytopenia could be a result of decreased bone marrow production, increased consumption, increased destruction, splenic sequestration or a combination of these causes. In this review, we have focused on some of the serious acquired causes of thrombocytopenia. There have been some significant advances in our understanding of the pathophysiology, diagnostic testing, and treatment of immune thrombocytopenia, heparin-induced thrombocytopenia, thrombotic thrombocytopenic purpura, and atypical hemolytic uremic syndrome over the past five years...
2018: F1000Research
https://www.readbyqxmd.com/read/29394550/-a-patient-with-basaloid-carcinoma-of-the-esophagus-removed-surgically-after-pre-operative-chemotherapy-who-developed-ttp-during-post-operative-chemotherapy
#8
Akemi Watanabe, Hiroyuki Fukunari, Yousuke Kawai, Makoto Aoki, Satoshi Saito, Yuya Umebayashi, Kenji Shitara, Tetsuji Hayashi, Gen Watanabe, Hiroshi Ajioka
A 63-year-old man presented with the chief complaint of an unpleasant feeling in the chest after a meal.Esophagogastroduodenoscopy revealed interminglement of ulcer infiltration type lesions and protruding lesions in the lower esophagus.A large type 1 protruding lesion was located mainly in the esophagogastric junction(EGJ)and it progressed towards the stomach.A hypertrophic and protruding lesion on the lower esophageal wall and a 6 cm tumor in the major axis of the fornix were observed on thoracic and abdominal CT, and an endocrine cell carcinoma or basaloid carcinoma were suggested after biopsy...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29387412/neurological-variability-in-chemotherapy-induced-posterior-reversible-encephalopathy-syndrome-associated-with-thrombotic-microangiopathy-case-reports-and-literature-review
#9
Chen Makranz, Salome Khutsurauli, Yosef Kalish, Ruth Eliahou, Luna Kadouri, John Moshe Gomori, Alexander Lossos
Posterior reversible encephalopathy syndrome (PRES) is a clinical syndrome characterized by headaches, seizures, a confusional state and visual disturbances associated with transient predominantly bilateral posterior white mater magnetic resonance imaging lesions. It is primarily reported in the setting of hypertension, acute renal failure, peripartum eclampsia, autoimmune disease, immunosuppression and chemotherapy. Thrombotic microangiopathy (TMA), including hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) has also been reported as potential PRES inducer...
January 2018: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/29357939/inherited-thrombotic-thrombocytopenic-purpura-mimicking-immune-thrombocytopenic-purpura-during-pregnancy-a-case-report
#10
Valter Romão de Souza, Ana Beatriz Cavalcante de Oliveira, Ana Maria Vanderlei, Amanda Queiroz da Mota Silveira Aroucha, Bruna Pontes Duarte, Aureli Nunes Machado, Lívia Netto Chaer, Cláudia Wanderley de Barros Correia, Maria da Conceição de Barros Correia, Manuela Freire Hazin Costa
BACKGROUND: Thrombotic thrombocytopenic purpura is a very rare hereditary blood deficiency disorder of ADAMTS13 (von Willebrand factor-cleaving protease) and a life-threatening thrombotic microangiopathy characterized by thrombocytopenia and microangiopathic hemolytic anemia. The deficiency in ADAMTS13 metalloprotease, which cleaves the von Willebrand factor, may be congenital or acquired. The congenital form is caused by inherited mutations in the ADAMTS13 gene. The diagnosis is challenging due to the nonspecific signs and symptoms, as well as the rarity of the disease...
January 22, 2018: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/29356300/pathophysiology-of-thrombotic-thrombocytopenic-purpura-and-hemolytic-uremic-syndrome
#11
Johanna A Kremer Hovinga, Silvan R Heeb, Magdalena Skowronska, Monica Schaller
Thrombotic microangiopathies are rare disorders characterized by the concomitant occurrence of severe thrombocytopenia, microangiopathic hemolytic anemia, and a variable degree of ischemic end organ damage. The latter particularly affects the brain, the heart and the kidneys. The primary forms, thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS), although in their clinical presentation often overlapping, have distinctive pathophysiologies. TTP is the consequence of a severe ADAMTS13 deficiency, immune-mediated due to circulating autoantibodies (iTTP), or caused by mutations in the ADAMTS13 gene (cTTP)...
January 22, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29352455/clinical-features-in-a-series-of-258-japanese-pediatric-patients-with-thrombotic-microangiopathy
#12
Akira Ashida, Hideki Matsumura, Toshihiro Sawai, Rika Fujimaru, Yuko Fujii, Akihiko Shirasu, Hyogo Nakakura, Kazumoto Iijima
BACKGROUND: Thrombotic microangiopathy (TMA) includes hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). This study examined the epidemiological characteristics of pediatric patients with TMA classified according to etiology. METHODS: The survey evaluated 258 Japanese pediatric patients diagnosed with TMA between 2012 and 2015. RESULTS: The primary diseases responsible for TMA were categorized as TTP (15 cases), Shiga toxin-producing Escherichia coli-associated HUS (STEC-HUS) (166 cases), atypical HUS (aHUS) (40 cases), and secondary TMA (27 cases)...
January 19, 2018: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29348275/microangiopathies-in-pregnancy
#13
Jailan Elayoubi, Kavitha Donthireddy, Divyesh Reddy Nemakayala
Thrombotic thrombocytopenic purpura (TTP) is a potentially reversible, life-threatening medical emergency. We present a case of a 21-year-old female with evidence of haemolytic anaemia based on the presence of positive markers of haemolysis. Negative Coomb's test, thrombocytopenia and placental infarcts raised suspicion for a thrombotic microangiopathy. She was diagnosed with TTP and managed with emergency plasma exchange. Her recovery was immediate.A presumptive diagnosis of TTP should be based on the presence of microangiopathic haemolytic anaemia with thrombocytopenia and plasma exchange should be initiated while complete work up is pending...
January 17, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29344548/recombinant-adamts-13-in-thrombotic-thrombocytopenic-purpura
#14
EDITORIAL
Marie Scully, Christopher Hibbard, Bruce Ewenstein
No abstract text is available yet for this article.
November 2017: Oncoscience
https://www.readbyqxmd.com/read/29340125/rare-presentation-of-refractory-thrombotic-thrombocytopenic-purpura-jejunal-stricture
#15
Prabath K Abeysundara, Inoshi Athukorala, K P C Dalpatadu, Karthiha Balendran, M D S A Dilrukshi, Gmo Fernando
Thrombotic thrombocytopenic purpura is a rare thrombotic disease characterized by episodes of thrombocytopenia and microangiopathic hemolytic anemia due to disseminated microvascular thrombosis. Thrombotic thrombocytopenic purpura was first described in 1924 by Moschowitz as a disease presenting with a pentad of signs and symptoms (anemia, thrombocytopenia, fever, hemiparesis and hematuria). Previous studies have described atypical manifestations of thrombotic thrombocytopenic purpura such as hemolysis, anemia and thrombosis...
October 1, 2017: International Journal of Hematology-oncology and Stem Cell Research
https://www.readbyqxmd.com/read/29337416/the-role-of-adamts-13-in-the-coagulopathy-of-sepsis
#16
REVIEW
Marcel Levi, Marie Scully, Mervyn Singer
The interaction between platelets and the vessel wall is mediated by various receptors and adhesive proteins, of which von Willebrand factor is most prominent. The multimeric size of von Willebrand factor is an important determinant of a more intense platelet-vessel wall interaction. and is regulated by the von Willebrand factor cleaving protease, ADAMTS-13. A deficiency in ADAMTS-13 leads to higher concentrations of ultra-large von Willebrand factor multimers and pathological platelet-vessel wall interactions, in its most typical and extreme form leading to thrombocytopenic thrombotic purpura (TTP), a thrombotic microangiopathy characterized by thrombocytopenia, non-immune haemolysis, and organ dysfunction...
January 16, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29304523/insights-into-3d-structure-of-adamts13-a-stepping-stone-towards-novel-therapeutic-treatment-of-thrombotic-thrombocytopenic-purpura
#17
Bogac Ercig, Kanin Wichapong, Chris P M Reutelingsperger, Karen Vanhoorelbeke, Jan Voorberg, Gerry A F Nicolaes
ADAMTS13 (A D: isintegrin A: nd M: etalloprotease with a T: hromboS: pondin type-1 motif, member 13: ) and von Willebrand factor (VWF) can be considered as scale weights which control platelet adhesion during primary haemostasis. In a very uncommon condition designated thrombotic thrombocytopenic purpura (TTP), functional absence of ADAMTS13 tips the balance toward VWF-mediated platelet adhesion in the microcirculation. TTP is associated with a high mortality and arises from either a congenital or acquired autoimmune deficiency of the plasma enzyme ADAMTS13...
January 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29296863/management-of-thrombotic-thrombocytopenic-purpura-without-plasma-exchange-the-jehovah-s-witness-experience
#18
James N George, Steven A Sandler, Joanna Stankiewicz
TTP in Jehovah's Witness patients has been managed successfully without PEX.This experience, plus new TTP treatments, may make it possible for patients who are not Jehovah's Witnesses to avoid PEX in the future.
November 14, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296854/cyclosporine-or-steroids-as-an-adjunct-to-plasma-exchange-in-the-treatment-of-immune-mediated-thrombotic-thrombocytopenic-purpura
#19
Spero R Cataland, Peter J Kourlas, Shangbin Yang, Susan Geyer, Leslie Witkoff, Haiwa Wu, Camila Masias, James N George, Haifeng M Wu
Although steroids are routinely used as an adjunct to plasma exchange (PEX) therapy in the treatment of immune-mediated thrombotic thrombocytopenic purpura (iTTP), limited data regarding their efficacy or effect on ADAMTS13 biomarkers are available. We report the results of a prospective, randomized study that compared the effectiveness of prednisone or cyclosporine (CSA) as adjuncts to PEX in the treatment of iTTP. A total of 26 of the planned 72 subjects were enrolled and treated from November 2007 until February 2014 before the study was halted after a planned interim analysis...
October 24, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296757/rituximab-prophylaxis-to-prevent-thrombotic-thrombocytopenic-purpura-relapse-outcome-and-evaluation-of-dosing-regimens
#20
John-Paul Westwood, Mari Thomas, Ferras Alwan, Vickie McDonald, Sylvia Benjamin, William A Lester, Gillian C Lowe, Tina Dutt, Quentin A Hill, Marie Scully
Acute antibody-mediated thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy with high morbidity and mortality. Rituximab is highly effective as prophylaxis in patients at risk of acute TTP relapse, but the ideal dosing regimen is unknown. A multicenter retrospective cohort study evaluated outcomes of patients given rituximab prophylaxis to prevent TTP relapse. Rituximab was given in 76 episodes to 45 patients (34 women and 11 men). Four once-per-week infusions of standard- (375 mg/m2 [24 episodes]), reduced- (200 mg [19 episodes]), and intermediate- (500 mg [17 episodes]) dose rituximab were given; in the remaining 16 episodes, patients received 100 to 1000 mg rituximab in 1 to 5 doses...
June 27, 2017: Blood Advances
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