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Thrombotic thrombocytopenia purpura

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https://www.readbyqxmd.com/read/28176472/suspected-atypical-haemolytic-uraemic-syndrome-in-two-post-partum-patients-with-foetal-death-in-utero-responding-to-eculizumab
#1
REVIEW
Justin Chua, Kathy Paizis, Simon Z He, Peter Mount
BACKGROUND: Atypical haemolytic uraemic syndrome (aHUS) is a rare condition with the triad of microangiopathic haemolytic anaemia, thrombocytopenia and acute kidney injury. Other conditions that present in a similar manner peri-partum include thrombotic thrombocytopaenic purpura, and pregnancy associated conditions including HELLP syndrome (haemolysis, elevated liver enzymes and low platelets), severe pre-eclampsia and less commonly acute fatty liver of pregnancy. CASE REPORTS: We describe two cases of suspected aHUS, who presented post-partum with foetal death-in-utero at 33 and 37 weeks respectively...
February 2017: Nephrology
https://www.readbyqxmd.com/read/28133771/thrombotic-thrombocytopenic-purpura-misdiagnosed-as-autoimmune-cytopenia-causes-of-diagnostic-errors-and-consequence-on-outcome-experience-of-the-french-thrombotic-microangiopathies-reference-centre
#2
Maximilien Grall, Elie Azoulay, Lionel Galicier, François Provôt, Alain Wynckel, Pascale Poullin, Steven Grange, Jean-Michel Halimi, Alexandre Lautrette, Yahsou Delmas, Claire Presne, Mohamed Hamidou, Stéphane Girault, Frédéric Pène, Pierre Perez, Tarik Kanouni, Amélie Seguin, Christiane Mousson, Dominique Chauveau, Mario Ojeda-Uribe, Virginie Barbay, Agnès Veyradier, Paul Coppo, Ygal Benhamou
Thrombotic thrombocytopenic purpura (TTP) has a devastating prognosis without adapted management. Sources of misdiagnosis need to be identified to avoid delayed treatment. We studied 84 patients with a final diagnosis of severe (<10%) acquired ADAMTS13 deficiency-associated TTP from our National database that included 423 patients, who had an initial misdiagnosis (20% of all TTP). Main diagnostic errors were attributed to autoimmune thrombocytopenia, associated (51%) or not (37%) with autoimmune hemolytic anemia...
January 30, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28033278/delayed-onset-of-posterior-reversible-encephalopathy-syndrome-in-a-case-of-scleroderma-renal-crisis-with-maintenance-hemodialysis-case-report-and-literature-review
#3
REVIEW
Ching-Yang Chen, Shin-Yuan Hung, Yi-Jer Lee, Yi-Chan Lin, Chu-Cheng Pai
INTRODUCTION: In some cases, scleroderma renal crisis (SRC) is not easily distinguishable from other thrombotic microangiopathies such as thrombotic thrombocytopenic purpura, especially when the presentation includes neurological or extra-renal manifestations. Here, we present a case of SRC who developed a rare neurotoxic complication, posterior reversible encephalopathy syndrome (PRES).A 36-year-old man with a history of diffuse cutaneous systemic sclerosis developed SRC and acute-on-chronic renal failure and ultimately required maintenance hemodialysis...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28011677/n-acetylcysteine-in-preclinical-mouse-and-baboon-models-of-thrombotic-thrombocytopenic-purpura
#4
Claudia Tersteeg, Jan Roodt, Walter J Van Rensburg, Charlotte Dekimpe, Nele Vandeputte, Inge Pareyn, Aline Vandenbulcke, Barbara Plaimauer, Seb Lamprecht, Hans Deckmyn, José A Lopez, Simon F De Meyer, Karen Vanhoorelbeke
Thrombotic thrombocytopenic purpura (TTP) is a microangiopathic disorder diagnosed by thrombocytopenia and hemolytic anemia, associated with a deficiency in von Willebrand factor (VWF)-cleaving protease ADAMTS13. Current treatment is based on plasma infusion for congenital TTP, or plasma exchange, often in combination with immunosuppressive agents, for acquired TTP. These treatment methods are not always effective; therefore, new treatment methods are highly necessary. N-acetylcysteine (NAC), an FDA-approved anti-mucolytic agent, is a possible new treatment strategy for TTP, as it was demonstrated to reduce disulfide bonds in VWF, thereby decreasing VWF multimers size and hence their prothrombotic potential...
February 23, 2017: Blood
https://www.readbyqxmd.com/read/27987277/eculizumab-refractory-thrombotic-thrombocytopenic-purpura-secondary-to-post-endoscopic-retrograde-cholangiopancreatography-pancreatitis-in-a-patient
#5
Faizan Malik, Naveed Ali, Irfan Ahsan, Ali Raza Ghani, Christian Fidler
Thrombotic thrombocytopenic purpura (TTP) is a rare multisystem microvascular disorder, which is characterized by pentad of thrombocytopenia, microangiopathic hemolytic anemia, and organ dysfunction due to occlusive thrombi. The proposed pathophysiology involves an imbalance between unusually large von Willebrand factor multimers and the cleaving protease ADAMTS13. Acute pancreatitis is a well-described consequence of TTP, but TTP secondary to acute pancreatitis is a rare phenomenon. We present a patient who developed TTP due to post-ERCP pancreatitis with hematologic, cardiovascular, pulmonary, and renal complications and is the first case of this kind...
2016: Journal of Community Hospital Internal Medicine Perspectives
https://www.readbyqxmd.com/read/27969556/severe-staphylococcus-aureus-endocarditis-presenting-as-peripartum-thrombotic-thrombocytopenic-purpura
#6
Kieran L Quinn, Mark Osmond, Mitesh Badiwala, Mathew Sermer, Stephen E Lapinsky
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening illness that occurs in both pregnant and non-pregnant women. Several other conditions can mimic the disease, which makes the diagnosis challenging. CASE: We describe a case of severe Staphylococcus aureus endocarditis that initially presented as peripartum TTP in a 39-year-old woman at 29+6 weeks' gestation. We give an overview of the diagnostic considerations and management of thrombocytopenia in pregnancy and review the literature related to TTP and peripartum infective endocarditis...
November 2016: Journal of Obstetrics and Gynaecology Canada: JOGC, Journal D'obstétrique et Gynécologie du Canada: JOGC
https://www.readbyqxmd.com/read/27930620/atypical-hemolytic-uremic-syndrome-ahus-essential-aspects-of-an-accurate-diagnosis
#7
REVIEW
Jeffrey Laurence, Hermann Haller, Pier Mannuccio Mannucci, Masaomi Nangaku, Manuel Praga, Santiago Rodriguez de Cordoba
Atypical hemolytic uremic syndrome (aHUS), a thrombotic microangiopathy (TMA), is a rare, life-threatening, systemic disease. When unrecognized or inappropriately treated, aHUS has a high degree of morbidity and mortality. aHUS results from chronic, uncontrolled activity of the alternative complement pathway, which activates platelets and damages the endothelium. Two-thirds of aHUS cases are associated with an identifiable complement-activating condition. aHUS is clinically very similar to the other major TMAs: Shiga toxin-producing Escherichia coli (STEC)-HUS, thrombotic thrombocytopenic purpura (TTP), and disseminated intravascular coagulation (DIC)...
November 2016: Clinical Advances in Hematology & Oncology: H&O
https://www.readbyqxmd.com/read/27898514/a-rare-combination-of-thrombotic-thrombocytopenic-purpura-and-antiphospholipid-syndrome
#8
Maya Viner, Irina Murakhovskaya
Thrombocytopenia, in the setting of microangiopathic hemolytic anemia and thrombotic events, is characteristic of both thrombotic thrombocytopenic purpura and primary antiphospholipid syndrome. Clinically, it is difficult to distinguish between these two syndromes. We present a 41-year-old woman with chronic, relapsing thrombotic thrombocytopenic purpura in the presence of antiphospholipid antibodies. She had clinical manifestations of antiphospholipid syndrome without meeting laboratory criteria of the Sydney classification system...
November 24, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27868334/consensus-on-the-standardization-of-terminology-in-thrombotic-thrombocytopenic-purpura-and-related-thrombotic-microangiopathies
#9
M Scully, S Cataland, P Coppo, J de la Rubia, K D Friedman, J Kremer Hovinga, B Lämmle, M Matsumoto, K Pavenski, E Sadler, R Sarode, H Wu
: Essentials An international collaboration provides a consensus for clinical definitions. This concerns thrombotic microangiopathies and thrombotic thrombocytopenic purpura (TTP). The consensus defines diagnosis, disease monitoring and response to treatment. Requirements for ADAMTS-13 are given. SUMMARY: Background Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) are two important acute conditions to diagnose. Thrombotic microangiopathy (TMA) is a broad pathophysiologic process that leads to microangiopathic hemolytic anemia and thrombocytopenia, and involves capillary and small-vessel platelet aggregates...
November 21, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27795524/exciting-investigator-initiated-clinical-trials
#10
Yoshitaka Miyakawa
In Japan, the media often announce investigator-initiated clinical trials (IIT). However, experts and reliable information remain limited. As most of the physicians involved do not have enough experience with the development of new medical drugs and devices, they need support from a contract research organization (CRO). The author recently managed two rituximab trials, one for immune thrombocytopenia (ITP) and the other for thrombotic thrombocytopenic purpura (TTP). It is hoped that the present lecture will be useful for investigators who are planning new IIT...
2016: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/27787280/acute-kidney-injury-in-a-patient-with-hemolytic-anemia-and-thrombocytopenia
#11
Michael Breunig, Miguel Lalama, Gabrielle Rivard, Deanne Kashiwagi, Lynn Cornell
Atypical hemolytic uremic syndrome (HUS) is clinically difficult to distinguish from HUS and thrombotic thrombocytopenic purpura. Atypical HUS results from dysregulation of complement activation causing thrombotic microangiopathy affecting multiple organ systems. Atypical HUS is associated with high morbidity and mortality, making early recognition and appropriate therapy necessary to improve patient outcomes.
November 2016: JAAPA: Official Journal of the American Academy of Physician Assistants
https://www.readbyqxmd.com/read/27781079/atypical-hemolytic-uremic-syndrome-secondary-to-lupus-nephritis-responsive-to-eculizumab
#12
Alexander G Raufi, Shruti Scott, Omar Darwish, Kevin Harley, Kanwarpal Kahlon, Sheetal Desai, Yuxin Lu, Minh-Ha Tran
Among the spectrum of disease manifestations associated with systemic lupus erythematosus, lupus nephritis is particularly concerning due to the potential for renal failure. This autoimmune attack may not, however, be limited to the kidney and is increasingly being recognized as a trigger for atypical Hemolytic Uremic Syndrome (aHUS). Atypical HUS falls under the spectrum of the thrombotic microangiopathies (TMAs) - a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and end organ damage...
September 28, 2016: Hematology Reports
https://www.readbyqxmd.com/read/27777394/systemic-lupus-erythematosus-presenting-as-refractory-thrombotic-thrombocytopenic-purpura-a-diagnostic-and-management-challenge-a-case-report-and-concise-review-of-the-literature
#13
REVIEW
Mohammad Abu-Hishmeh, Alamgir Sattar, Fnu Zarlasht, Mohamed Ramadan, Aisha Abdel-Rahman, Shante Hinson, Caroline Hwang
BACKGROUND Thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathic (TMA) syndromes, caused by severely reduced activity of the vWF-cleaving protease ADAMTS13. Systemic lupus erythematosus (SLE), on the other hand, is an autoimmune disease that affects various organs in the body, including the hematopoietic system. SLE can present with TMA, and differentiating between SLE and TTP in those cases can be very challenging, particularly in patients with no prior history of SLE. Furthermore, an association between these 2 diseases has been described in the literature, with most of the TTP cases occurring after the diagnosis of SLE...
October 25, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27734437/thrombotic-microangiopathy-syndrome-in-a-basic-underwater-demolition-seal-student
#14
Daniel Croom, Heather Tracy
Thrombotic microangiopathy (TMA) syndromes represent a spectrum of illnesses that share common clinical and pathologic features of microangiopathic hemolytic anemia, thrombocytopenia, and organ injury from pathologic small-vessel thrombosis. At least nine primary TMA syndromes have been described and classified based on common probable etiologies, diagnostic criteria, and treatments. The most recognized of the TMA syndromes include thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS)...
December 0: Journal of Special Operations Medicine: a Peer Reviewed Journal for SOF Medical Professionals
https://www.readbyqxmd.com/read/27717013/increased-troponin-i-is-associated-with-fatal-outcome-in-acquired-thrombotic-thrombocytopenic-purpura
#15
Jason Brazelton, Robert A Oster, Brandi McCleskey, Jessica Fuller, Jill Adamski, Marisa B Marques
Thrombotic thrombocytopenic purpura (TTP) has >90% mortality without therapeutic plasma exchange (TPE). Despite TPE, approximately 10% of patients still die, presumably from cardiac ischemia. We sought clinical or laboratory parameters associated with death by reviewing the records of all patients hospitalized with acquired TTP in our institution for 10 years, and collect demographics and results for hemoglobin, platelet count, creatinine, lactate dehydrogenase, transaminases, total bilirubin, creatinine kinase (CK), CK-MB, and troponin I...
September 26, 2016: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/27658429/biophysical-tools-to-assess-the-interaction-of-pf4-with-polyanions
#16
Mihaela Delcea, Andreas Greinacher
The antigen in heparin-induced thrombocytopenia (HIT) is expressed on platelet factor 4 (PF4) when PF4 complexes with polyanions. In recent years, biophysical tools (e. g. circular dichroism spectroscopy, atomic force microscopy, isothermal titration calorimetry, x-ray crystallography, electron microscopy) have gained an important role to complement immunological and functional assays for better understanding the interaction of heparin with PF4. This allowed identification of those features that make PF4 immunogenic (e...
October 28, 2016: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/27648610/thrombotic-thrombocytopenic-purpura-and-atypical-hemolytic-uremic-syndrome-microangiopathy-in-pregnancy
#17
Marie Scully
Thrombotic thrombocytopenic purpura (TTP) and atypical hemolytic uremic syndrome (aHUS) are thrombotic microangiopathies (TMAs) that can present in pregnancy. The presentation can be with typical microangiopathic features and thrombocytopenia, but there is also a significant risk of in-utero fetal loss. TTP presents most commonly in the third trimester and aHUS in the postpartum period. On presumptive diagnosis, plasma exchange should be started and ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity measured...
October 2016: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/27628419/acute-myocardial-infarction-caused-by-thrombotic-microangiopathy-complicated-with-myelodysplastic-syndrome
#18
Tsukasa Oshima, Masayasu Ikutomi, Hiroki Shinohara, Jumpei Ishiwata, Keiko Fukino, Toshihiro Amaki, Fumitaka Nakamura
Thrombotic microangiopathy (TMA) is a rare but lethal multisystem disease characterized by peripheral thrombocytopenia, microangiopathic hemolytic anemia, fever, and various stages of renal and neurological dysfunctions.(1,2)) The causes of TMA are mainly thrombotic thrombocytopenic purpura (TTP) or hemolytic-uremic syndrome (HUS), and cases of TMA related to myelodysplastic syndrome (MDS) are quite rare. Herein, we report a case of acute myocardial infarction (AMI) caused by TMA which is strongly suspected to have a relationship to MDS, and discuss the treatment of our patient who needed antiplatelet or anticoagulant therapy after AMI, while on the other hand, had pancytopenia and a bleeding event due to MDS...
September 28, 2016: International Heart Journal
https://www.readbyqxmd.com/read/27617501/c3-ch50-ratio-as-a-proposed-composite-marker-for-eculizumab-monitoring-in-atypical-hemolytic-uremic-syndrome-preliminary-results
#19
Kheir Eddine Kerboua, Fatma Haiba, Djamila Batouche
Treatment of atypical hemolytic uremic syndrome (aHUS) by the complement C5 inhibitor eculizumab (Soliris®) is highly effective but unfortunately, associated with an economic pressure on the health care systems even in high incomes countries. Despite spacing infusions has been proposed as the unique solution to minimize this economic impact, no reliable laboratory assays are available to tailor such therapy optimization. We aimed to propose and evaluate a complement composite marker for eculizumab efficacy monitoring in aHUS...
September 12, 2016: Journal of Immunoassay & Immunochemistry
https://www.readbyqxmd.com/read/27609730/an-improbable-and-unusual-case-of-thrombotic-thrombocytopenia-purpura
#20
Jaymon Patel, Preeti Patel, Zohair Ahmed
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening medical emergency which may be difficult to recognize given the wide spectrum in which it presents. A delay in treatment may be catastrophic as untreated cases of TTP have a mortality rate exceeding 90%. Given the high fatality rate of untreated TTP and its range of presenting symptoms, we present our unusual case of TTP in a post-splenectomy patient with early treatment and positive outcome. This case describes a 54-year-old female who presented with hematuria and gingival bleeding, followed by the development of a bilateral lower extremity petechial rash...
2016: Journal of Community Hospital Internal Medicine Perspectives
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