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Thrombotic thrombocytopenic purpura

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https://www.readbyqxmd.com/read/29228282/proteolytic-inactivation-of-adamts13-by-plasmin-in-human-plasma-risk-of-thrombotic-thrombocytopenic-purpura
#1
Yongchol Shin, Haruki Miyake, Kenshi Togashi, Ryuichi Hiratsuka, Kana Endou-Ohnishi, Yasutada Imamura
Thrombotic thrombocytopenic purpura (TTP) is caused by inactivation of a von Willebrand factor (VWF)-cleaving enzyme, ADAMTS13, which leads to platelet-rich thrombi comprising unusually large VWF multimers. We have found that ADAMTS13 can bind to the inactivated form of plasmin. In addition, plasmin cleaves purified ADAMTS13 into several fragments and inactivates it. Hence, we hypothesized that activation of plasminogen to plasmin becomes a new-onset factor for TTP due to ADAMTS13 inactivation. Plasmin was added exogenously or activated from plasminogen by streprokinase addition in HP...
December 7, 2017: Journal of Biochemistry
https://www.readbyqxmd.com/read/29222940/an-open-conformation-of-adamts13-is-a-hallmark-of-acute-acquired-thrombotic-thrombocytopenic-purpura
#2
E Roose, A S Schelpe, B S Joly, M Peetermans, P Verhamme, J Voorberg, A Greinacher, H Deckmyn, S F De Meyer, P Coppo, A Veyradier, K Vanhoorelbeke
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (aTTP) is an autoimmune disorder characterized by absent ADAMTS13 activity and presence of anti-ADAMTS13 autoantibodies. Recently it was shown that ADAMTS13 adopts a folded or an open conformation. OBJECTIVES: As conformational changes in self-antigens play a role in the pathophysiology of different autoimmune diseases, we hypothesized that the conformation of ADAMTS13 changes during acute aTTP. METHODS: Antibodies recognizing cryptic epitopes in the spacer domain were generated...
December 9, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29222317/thrombocytopenia-in-hospitalized-patients-approach-to-the-patient-with-thrombotic-microangiopathy
#3
REVIEW
Marie Scully
Thrombotic microangiopathies (TMAs), specifically, thrombotic thrombocytopenic purpura (TTP) and complement-mediated hemolytic uremic syndrome (CM-HUS) are acute life-threatening disorders that require prompt consideration, diagnosis, and treatment to improve the high inherent mortality and morbidity. Presentation is with microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) and variable organ symptoms resulting from microvascular thrombi. Neurological and cardiac involvement is most common in TTP and associated with poorer prognosis and primarily renal involvement in CM-HUS...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222314/plasma-exchange-in-thrombotic-microangiopathies-tmas-other-than-thrombotic-thrombocytopenic-purpura-ttp
#4
REVIEW
Jeffrey L Winters
Thrombotic microangiopathies (TMAs) are a diverse group of disorders that are characterized by common clinical and laboratory features. The most commonly thought-of TMA is thrombotic thrombocytopenic purpura (TTP). Because of the marked improvement in patient mortality associated with the use of therapeutic plasma exchange (TPE) in TTP, this therapy has been applied to all of the TMAs. The issue, however, is that the pathophysiology varies and in many instances may represent a disorder of the endothelium and not the blood; in some cases, the pathophysiology is unknown...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29222249/thrombocytopenia-in-pregnancy
#5
REVIEW
Douglas B Cines, Lisa D Levine
Thrombocytopenia develops in 5% to 10% of women during pregnancy or in the immediate postpartum period. A low platelet count is often an incidental feature, but it might also provide a biomarker of a coexisting systemic or gestational disorder and a potential reason for a maternal intervention or treatment that might pose harm to the fetus. This chapter reflects our approach to these issues with an emphasis on advances made over the past 5 to 10 years in understanding and managing the more common causes of thrombocytopenia in pregnancy...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29219891/thrombotic-thrombocytopenic-purpura-in-a-child-treated-for-acute-lymphoblastic-leukemia-case-report-and-review-of-literature
#6
Francesco De Leonardis, Roberta Koronica, Rosa Maria Daniele, Nicola Santoro
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by deficiency of von Willebrand factor-cleaving protease ADAMTS13. Large von Willebrand multimer formation and intravascular platelet aggregation affecting the arterioles and capillaries can result in death unless early treatment is administered. We report on the case of a child with TTP associated with a human herpes virus type-6 infection occurring during chemotherapy for acute lymphoblastic leukemia who was effectively treated by fresh frozen plasma infusions and antiviral therapy...
December 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/29206764/pet-ct-in-critically-ill-patients-diagnosing-the-unsuspected
#7
Romain Fort, Stanislas Ledochowski, Arnaud Friggeri
OBJECTIVE: Quick identification of septic source is fundamental in patients with severe sepsis of unknown origin. The purpose of this case report was to assess the benefit and feasibility of an early PET-CT in critically ill patients with undiagnosed sepsis. DATA SOURCES: Clinical observations of two patients. STUDY SELECTION: Case reports. DATA EXTRACTION: Data extracted from medical records, after patient's consent...
December 4, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/29196200/incidental-thrombotic-thrombocytopenic-purpura-during-acute-ischemic-stroke-and-thrombolytic-treatment
#8
İbrahim Acır, Hacı Ali Erdoğan, Vildan Yayla, Nilay Taşdemir, Murat Çabalar
BACKGROUND: Intravenous tissue plasminogen activator (IV tPA) was shown to be an effective treatment for acute ischemic stroke (AIS). According to stroke guidelines, there is no need to wait for the complete blood count (CBC) and coagulation test results before application of IV alteplase if there is no suspected coagulation disorder. In this study, a patient with AIS and thrombotic thrombocytopenic purpura (TTP) symptoms during thrombolytic treatment was presented. CASE: A 33-year-old male patient was admitted at the 2...
November 28, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/29187254/acute-disseminated-melioidosis-giving-rise-to-pneumonia-and-renal-abscesses-complicated-with-thrombotic-thrombocytopenic-purpura-in-a-post-partum-woman-a-case-report
#9
Piyumi Sachindra Alwis Wijewickrama, Rohini Weerakoon
BACKGROUND: Melioidosis is an established endemic infection in Sri Lanka, caused by Burkholderia pseudomallei, a gram negative bacterium distributed in saprophytes in soil and surface water. Main mode of transmission is via percutaneous inoculation. Pneumonia is the most common presentation in acute disease. CASE PRESENTATION: We report a 33 year old previously healthy Sinhalese female with an occupational exposure to surface water in paddy fields, who was on postpartum day 6 following an uncomplicated pregnancy and delivery via an elective caesarian section...
November 29, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/29185285/posterior-reversible-encephalopathy-syndrome-pres-associated-with-thrombotic-thrombocytopenic-purpura-in-a-systemic-lupus-erythematosus-patient
#10
Szilvia Szamosi, Nóra Bodnár, Boglárka Brugós, Tibor Hortobágyi, Gábor Méhes, Zoltán Szabó, Edit Végh, Ágnes Horváth, Zoltán Szekanecz, Attila Szűcs, Gabriella Szűcs
No abstract text is available yet for this article.
November 2017: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/29178379/successful-treatment-of-thrombotic-microangiopathy-associated-to-dengue-infection-a-case-report-and-literature-review
#11
John Fredy Nieto-Ríos, María Fernanda Álvarez Barreneche, Sara Catalina Penagos, Diana Carolina Bello Márquez, Lina Maria Serna-Higuita, Isabel Cristina Ramírez Sánchez
Dengue infection has been associated with multiple renal complications, including glomerulonephritis, acute tubular necrosis, tubulointerstitial nephritis, and thrombotic microangiopathy (TMA); the last one being a rare complication of dengue, with only a few reported cases. TMA associated with dengue can be explained by an alteration in the activity of the enzyme ADAMTS13, leading to thrombotic thrombocytopenic purpura; or it can be secondary to direct or indirect endothelial injury by the virus, which leads to hemolytic uremic syndrome...
November 27, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/29150875/treatment-of-acquired-thrombotic-thrombocytopenic-purpura-in-the-u-s-remains-heterogeneous-current-and-future-points-of-clinical-equipoise
#12
Marshall A Mazepa, Jay S Raval, Mark E Brecher, Yara A Park
BACKGROUND: The purpose of this survey was to describe current practices in the U.S. for treatment of acquired Thrombotic Thrombocytopenic Purpura (TTP), compare these with prior U.S. and current Canadian practices, and identify areas of clinical equipoise. STUDY DESIGN AND METHODS: A research team member administered the survey by telephone. Questions included an estimate of the annual patient volume treated, apheresis and medical therapy practices for acquired TTP...
November 18, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/29130141/sj%C3%A3-gren-s-syndrome-initially-presented-as-thrombotic-thrombocytopenic-purpura-in-a-male-patient-a-case-report-and-literature-review
#13
REVIEW
Xiaohan Xu, Tienan Zhu, Di Wu, Lu Zhang
Thrombotic thrombocytopenic purpura (TTP) is a potentially lethal multisystem disorder which could be caused by autoimmune diseases. However, the concomitant occurrence of TTP and Sjögren's syndrome (SS) is an extremely uncommon scenario, especially in male patients. A 56-year-old Chinese male was admitted for the appearance of diffuse ecchymosis. Then he gradually developed transient slurred speech, progressive confusion, agitation, extremity weakness, and fever. Laboratory investigations suggested anemia, thrombocytopenia, significantly increased lactic dehydrogenase, schistocytes in peripheral blood smear, and a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 (ADAMTS13) activity deficiency with high inhibitor titers...
November 13, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29124560/maintenance-treatment-using-the-purine-synthesis-inhibitor-mizoribine-in-a-patient-with-relapsing-thrombotic-thrombocytopenic-purpura
#14
Tetsuya Kawamura, Joichi Usui, Kei Nagai, Kentaro Sakai, Itaru Ebihara, Kunihiro Yamagata
Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening disease. Although plasma exchange (PE) therapy and corticosteroids are standard remission induction and maintenance therapies, some patients are easily refractory and frequently relapse under treatment with this therapy, and require additional treatment. However, there are limited data about additional treatment interventions. We report a case of 56-year-old man who was hospitalized for fever, general fatigue and hemoglobinuria. Owing to the symptoms and the laboratory findings of hemolysis, he was diagnosed with TTP...
November 9, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/29123569/diagnostic-dilemma-severe-thrombotic-microangiopathy-in-pregnancy
#15
Sarah Birkhoelzer, Alexandra Belcher, Helen Peet
A diagnostic dilemma occurred when thrombotic microangiopathy developed during pregnancy. The diagnostic criteria of thrombotic microangiopathy include thrombocytopenia (platelets <100) and microangiopathic haemolytic anaemia (including thrombotic thrombocytopenic purpura and haemolytic-uraemic syndrome). An urgent interdisciplinary approach is required to treat thrombotic microangiopathy in pregnancy to differentiate between thrombotic microangiopathy and HELLP syndrome (haemolysis, elevated liver enzymes, low platelets)...
November 2017: Journal of the Intensive Care Society
https://www.readbyqxmd.com/read/29121438/platelet-rescue-by-macrophage-depletion-in-obese-adamts13-deficient-mice-at-risk-for-thrombotic-thrombocytopenic-purpura
#16
Lotte Geys, Elien Roose, Ilse Scroyen, Hanspeter Rottensteiner, Claudia Tersteeg, Marc F Hoylaerts, Karen Vanhoorelbeke, H Roger Lijnen
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is caused by the absence of ADAMTS13 activity. Thrombocytopenia is presumably related to the formation of microthrombi rich in von Willebrand Factor (VWF) and platelets. Obesity may be a risk factor for TTP; it is associated with abundance of macrophages that may phagocytose platelets. OBJECTIVES: To evaluate the role of obesity and ADAMTS13 deficiency in TTP, and to establish whether macrophages contribute to thrombocytopenia...
November 9, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29105831/the-importance-of-clinical-judgment-for-the-diagnosis-of-thrombotic-thrombocytopenic-purpura
#17
EDITORIAL
James N George
No abstract text is available yet for this article.
November 2017: Transfusion
https://www.readbyqxmd.com/read/29105485/thrombotic-thrombocytopenic-purpura-presenting-as-a-severe-peripartum-cardiogenic-shock-role-of-myocardial-biopsy-and-assist-device-for-diagnosis-and-resuscitation
#18
Sébastien Champion, Dominique Belcour, Bernard Alex Gaüzère
We describe the case of a peripartum thrombotic thrombocytopenic purpura with fulminant cardiogenic shock treated with extracorporeal life support. Thrombotic thrombocytopenic purpura should be considered in the case of thrombotic microangiopathy with several or severe organ involvement and needs emergent treatment with plasmapheresis (with or without rituximab). In the case of cardiac involvement, aggressive treatment should be considered given the high mortality and the potential complete recovery.
November 1, 2017: European Heart Journal. Acute Cardiovascular Care
https://www.readbyqxmd.com/read/29095327/cerebral-venous-sinus-thrombosis-due-to-low-molecular-weight-heparin-induced-thrombocytopenia
#19
Ezequiel Gleichgerrcht, Ming Y Lim, Tanya N Turan
INTRODUCTION: Heparin-induced thrombocytopenia (HIT) is an immune-mediated complication of heparin exposure. A limited number of studies have reported cerebral venous sinus thrombosis (CVST) as the presenting thrombotic event induced by HIT, only one of which occurred with exposure to low-molecular-weight heparin (LMWH), with death as outcome. Here, we present a unique case of LMWH-induced HIT leading to CVST but resulting in good clinical outcome. CASE REPORT: A 52-year-old woman received subcutaneous LMWH for deep vein thrombosis prophylaxis while in rehabilitation following kyphoplasty for spinal fracture related to recent trauma...
November 2017: Neurologist
https://www.readbyqxmd.com/read/29094300/what-is-behind-a-relapse-of-thrombotic-thrombocytopenic-purpura
#20
Marco Capecchi, Andrea Artoni, Maria Domenica Cappellini, Giovanna Graziadei
No abstract text is available yet for this article.
November 2, 2017: Internal and Emergency Medicine
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