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Thrombotic thrombocytopenic purpura

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https://www.readbyqxmd.com/read/29040872/highly-elevated-plasma-level-of-von-willebrand-factor-accelerates-the-formation-of-platelet-thrombus-under-high-shear-stress-in-plasma-with-deficient-adamts13-activity
#1
Hideo Yagi, Naoko Yamaguchi, Yasuaki Shida, Mitsuhiko Sugimoto, Kazuo Tubaki, Yoshihiro Fujimura, Masanori Matsumoto
Upshaw-Schulman syndrome (USS) is a thrombo-hemorrhagic disease caused by congenital deficiency of ADAMTS13 due to ADAMTS13 gene mutations. USS is characterized by repeated episodes of thrombocytopenia and microangiopathic hemolytic anemia that respond dramatically to infusions of fresh frozen plasma. There are two phenotypic expressions of USS: one is the early-onset type and the other, the late-onset type, is asymptomatic during childhood with the first bout of thrombotic thrombocytopenic purpura (TTP) developing after adolescence or during adulthood...
October 10, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/29038197/obstructive-coronary-artery-disease-in-patient-with-acute-thrombotic-thrombocytopenic-purpura
#2
Szymon L Wiernek, Xuming Dai
Thrombotic thrombocytopenic purpura (TTP) affects essentially all organ systems. Myocardial injury in TTP is often attributed to microthrombi formation. We present the first case report in the literature of an acute TTP patient with concomitant obstructive coronary artery disease (CAD) and acute myocardial infarction who underwent successful percutaneous coronary intervention (PCI). A 70-year-old female patient who was diagnosed with acute TTP required plasma exchange. The patient also experienced episodes of angina pectoris, elevated cardiac enzymes and global ST segment depressions on ECG...
October 15, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28983021/sayani-fa-abrams-cs-how-i-treat-refractory-thrombotic-thrombocytopenic-purpura-blood-2015-125-25-3860-3867
#3
(no author information available yet)
No abstract text is available yet for this article.
October 5, 2017: Blood
https://www.readbyqxmd.com/read/28981198/amplified-endogenous-plasmin-activity-resolves-acute-thrombotic-thrombocytopenic-purpura-in-mice
#4
Claudia Tersteeg, Bérangère S Joly, Ann Gils, Roger Lijnen, Hans Deckmyn, Paul J Declerck, Barbara Plaimauer, Paul Coppo, Agnès Veyradier, Coen Maas, Simon F De Meyer, Karen Vanhoorelbeke
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is an acute life-threatening pathology, caused by occlusive von Willebrand factor (VWF)-rich microthrombi that accumulate in absence of ADAMTS13. We previously demonstrated that plasmin can cleave VWF and that plasmin is generated in patients during acute TTP. However, the exact role for plasmin in TTP remains unclear. OBJECTIVES: Investigate if endogenous plasmin-mediated proteolysis of VWF can influence acute TTP episodes...
October 5, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28978852/new-developments-in-treatment-modalities-of-thrombotic-thrombocytopenic-purpura
#5
Masanori Matsumoto
Although thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease, appropriate diagnosis and treatment result in the higher survival rate of >80%. TTP is usually suspected with thrombocytopenia and hemolytic anemia and is confirmed by a reduced activity of a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13 (ADAMTS13) <10%. TTP is classified as acquired if a patient tests positive for anti-ADAMTS13 autoantibodies, and as congenital if ADAMTS13 gene abnormalities are identified...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28975130/multiple-myeloma-as-the-underlying-cause-of-thrombotic-microangiopathy-leading-to-acute-kidney-injury-revisiting-a-very-rare-entity
#6
Savneek Chugh, Asim Kichloo, Firas Jafri, Liga Yusvirazi, Robert Lerner
Thrombotic microangiopathy (TMA) describes a pathological process of microvascular thrombosis, consumptive thrombocytopenia, and microangiopathic hemolytic anemia, leading to end-organ ischemia and infarction, affecting particularly the kidney and brain. TMA is a pathological feature of a number of clinical disorders including thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, and atypical hemolytic uremic syndrome. Rare but important, TMA may also occur in malignancy, connective tissue disease, malignant hypertension, and renal transplantation (rejection or drug toxicity)...
July 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28969551/aptamer-oligonucleotides-as-potential-therapeutics-in-hematologic-diseases
#7
Weibin Li, Meng Zhao, Kaiyu Wang, Huihui Yan, XIaopeng Lan
Aptamers are single-stranded DNA or RNA oligonucleotides generated by a novel in vitro selection technique termed Systematic Evolution of Ligands by Exponential Enrichment (SELEX). During the past two decades, various aptamer drugs have been developed and many of them have entered into clinical trials. In the present review, we focus on aptamers as potential therapeutics for hematological diseases, including anemia of chronic inflammation (ACI) and anemia of chronic disease (ACD), hemophilia, thrombotic thrombocytopenic purpura (TTP) or VWD type-2B, and sickle cell disease (SCD), in particular, those that have entered into clinical trials...
October 2, 2017: Mini Reviews in Medicinal Chemistry
https://www.readbyqxmd.com/read/28940604/asfa-category-iv-becomes-category-i-idiopathic-thrombotic-thrombocytopenic-purpura-in-a-patient-with-presumed-gemcitabine-induced-thrombotic-microangiopathy
#8
Peter G Bittar, Myles S Nickolich, Oluwatoyosi A Onwuemene
In the implementation of American Society for Apheresis national guidelines, the decision for therapeutic plasma exchange may be confounded by a clinical presentation that fits both a Category I and IV designation. We report the case of a 45-year-old female who presented with concern for a Category IV disorder, gemcitabine-induced thrombotic microangiopathy, and was ultimately diagnosed with a Category I disorder, idiopathic thrombotic thrombocytopenic purpura. This case highlights the importance of ruling out idiopathic TTP by a thorough evaluation for ADAMTS13 activity and inhibitor, even when an alternate thrombotic microangiopathy diagnosis may be likely...
September 23, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28940540/successful-kidney-transplantation-in-a-patient-with-congenital-thrombotic-thrombocytopenic-purpura-upshaw-schulman-syndrome
#9
Hasan Fattah, Dhiren Kumar, James N George, H Davis Massey, Anne L King, Kenneth D Friedman, Gaurav Gupta
BACKGROUND: Congenital thrombotic thrombocytopenic purpura (TTP) may not be recognized until organ failure related to the microvascular thrombosis occurs. Kidney failure may be the initial presenting clinical feature. Kidney transplantation has been contraindicated because of the assumption that the continuing microvascular thrombosis will cause inevitable graft failure. CASE REPORT: We report a 48-year-old nulliparous woman who presented with end-stage kidney disease that was attributed to hypertension...
September 20, 2017: Transfusion
https://www.readbyqxmd.com/read/28932128/a-suspected-case-of-autoinduction-of-voriconazole-metabolism-in-a-patient-with-cerebral-aspergillosis
#10
Martin J Ferguson, Maria L Randles, Declan G de Freitas
OBJECTIVE: This study aims to report a case of accelerated metabolism of voriconazole in a patient with cerebral aspergillosis. CASE SUMMARY: A 36-year-old woman developed cerebral aspergillosis after immunosuppressive treatment for suspected atypical hemolytic uremic syndrome/thrombotic thrombocytopenic purpura. She was treated with voriconazole using therapeutic drug monitoring to guide dosing. After an initial high level, her dose was reduced, but over the following weeks, she required several dose increases in order to achieve a voriconazole level within the target range...
2017: Drug, Healthcare and Patient Safety
https://www.readbyqxmd.com/read/28904490/dengue-and-thrombotic-thrombocytopenic-purpura
#11
Viroj Wiwanitkit
No abstract text is available yet for this article.
August 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28904488/thrombotic-thrombocytopenic-purpura-or-disseminated-intravascular-coagulation
#12
Ashok Kumar Pannu, Atul Saroch
No abstract text is available yet for this article.
August 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28884355/influenza-associated-thrombotic-microangiopathies
#13
REVIEW
Martin Bitzan, Jakub Zieg
Thrombotic microangiopathy (TMA) refers to phenotypically similar disorders, including hemolytic uremic syndromes (HUS) and thrombotic thrombocytopenic purpura (TTP). This review explores the role of the influenza virus as trigger of HUS or TTP. We conducted a literature survey in PubMed and Google Scholar using HUS, TTP, TMA, and influenza as keywords, and extracted and analyzed reported epidemiological and clinical data. We identified 25 cases of influenza-associated TMA. Five additional cases were linked to influenza vaccination and analyzed separately...
September 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28883277/siblings-with-congenital-thrombotic-thrombocytopenic-purpura
#14
Yasutomo Funakoshi, Masahiko Okada, Masanori Matsumoto, Koichi Kokame, Hiroyuki Moriuchi
Congenital thrombotic thrombocytopenic purpura (TTP) is a rare hereditary deficiency of ADAMTS13 (von Willebrand factor-cleaving protease) characterized by thrombocytopenia and microangiopathic hemolytic anemia. The spectrum of the clinical phenotype is wide, ranging from asymptomatic episodes of thrombocytopenia to life-threatening multiorgan failure. Reportedly, some patients develop isolated thrombocytopenia during childhood. We herein report sibling cases of congenital TTP. An 11-year-old boy with thrombocytopenia accompanied by influenza virus infection was referred to our hospital...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28878919/from-anemia-to-polycythemia-in-4-weeks
#15
Omer A Hassan, Melissa Y Y Moey, Christos N Papageorgiou
Primary polycythemia (PCV) may coexist in otherwise asymptomatic patients particularly in the presence of unsuspecting conditions such as Thrombotic thrombocytopenic purpura (TTP). In presumed "idiopathic TTP," autoimmune conditions such as rheumatoid arthritis (RA) should be investigated as a possible etiology for TTP. Standardization of targeted therapy with immunomodulatory agents may be recommended for this subset of patients.
September 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28874058/lacosamide-induced-thrombotic-thrombocytopenic-purpura
#16
Dènahin Hinnoutondji Toffa, Sepehr Mehrpouyan, Benjamin Rioux-Masse, Dang Khoa Nguyen
No abstract text is available yet for this article.
September 1, 2017: Annals of Pharmacotherapy
https://www.readbyqxmd.com/read/28860441/-a-case-of-merkel-cell-carcinoma-complicated-with-severe-thrombocytopenia-treated-with-carboplatin-etoposide-regimen-after-surgery
#17
Yuko Saito, Takayoshi Kiba, Toru Takahashi, Hiroyuki Hirakawa, Miho Saito, Chiaki Otomo, Miho Sato, Yoshiteru Watanabe, Masanobu Sakaguchi, Risako Kadota, Naoya Noguchi, Takahiro Suzuki, Toshikazu Awataguchi, Fumi Shoji, Nobuo Ota
Thrombocytopenia is often caused by myelosuppression during chemotherapy. However, when platelet transfusions are required, pathological conditions such as idiopathic thrombocytopenic purpura(ITP)and thrombotic thrombocytopenic purpura( TTP)also occur. We report a case of Merkel cell carcinoma complicated with severe thrombocytopenia treated with carboplatin/etoposide regimen after surgery. The patient's platelet count did not increase in spite of platelet transfusions. However, the platelet count increased after steroid treatment was chosen under the diagnosis of ITP...
August 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/28858843/using-plasma-exchange-to-successfully-manage-thyrotoxicosis-in-a-patient-with-possible-antithyroid-drug-related-thrombotic-thrombocytopenic-purpura
#18
G Tazegul, T S Ogut, H Bozoglan, O Dogan, N Yilmaz, T Ulas, O Salim, R Sari, H A Altunbas, M K Balci
OBJECTIVE: Thrombotic thrombocytopenic purpura (TTP) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenic purpura, neurologic abnormalities, fever, and renal insufficiency. The association or co-existence of thyrotoxicosis or antithyroid drugs with TTP has not been previously reported. Subject and Results. Herein, we present a 54-year-old female patient newly diagnosed with toxic multinodular goiter accompanying with TTP, possibly triggered by either thyrotoxicosis or antithyroid drugs...
July 1, 2017: Endocrine Regulations
https://www.readbyqxmd.com/read/28833243/a-novel-homozygous-frameshift-mutation-in-exon-7-of-the-adamts13-gene-in-a-patient-with-congenital-thrombotic-thrombocytopenic-purpura-from-india-a-case-report
#19
Sneha Yadav, Shrimati Shetty, Bipin Kulkarni
BACKGROUND: Thrombotic thrombocytopenia purpura (TTP) is a rare and life-threatening thrombotic microangiopathy characterized by thrombocytopenia and microangiopathic hemolytic anemia. It is caused by deficiency of ADAMTS13 metalloprotease, which cleaves ultra-large von Willebrand factor into smaller functional units. TTP may be congenital or acquired, and the congenital form is caused by inherited mutations in the ADAMTS13 gene, leading to deficiency of protein or reduced protein activity...
August 21, 2017: Transfusion
https://www.readbyqxmd.com/read/28826581/uncommon-causes-of-cerebral-microbleeds
#20
REVIEW
Nariman Noorbakhsh-Sabet, Varun Chandi Pulakanti, Ramin Zand
BACKGROUND: Cerebral microbleeds (CMBs) are small and round perivascular hemosiderin depositions detectable by gradient echo sequences or susceptibility-weighted imaging. Cerebral microbleeds are common among patients with hypertension, cerebral ischemia, or cerebral amyloid angiopathy. In this article, we describe uncommon causes of CMBs. METHODS: We searched Pubmed with the keyword CMBs for relevant studies and looked for different uncommon causes of CMBs. RESULTS: CMBs have several uncommon etiologies including posterior reversible encephalopathy syndrome, infective endocarditis, brain radiation therapy, cocaine abuse, thrombotic thrombocytopenic purpura, traumatic brain injury, intravascular lymphomatosis or proliferating angio-endotheliomatosis, moyamoya disease, sickle cell anemia/β-thalassemia, cerebral autosomal dominant arteriopathy subcortical infarcts, and leukoencephalopathy (CADASIL), genetic syndromes, or obstructive sleep apnea...
August 17, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
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