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thrombocytopenic purpura

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https://www.readbyqxmd.com/read/28521259/von-willebrand-factor-and-its-cleaving-protease-adamts13-balance-in-coronary-artery-vessels-lessons-learned-from-thrombotic-thrombocytopenic-purpura-a-narrative-review
#1
REVIEW
Nuccia Morici, Silvia Cantoni, Francesco Panzeri, Alice Sacco, Chiara Rusconi, Miriam Stucchi, Fabrizio Oliva, Marco Cattaneo
BACKGROUND: Deficiency of the von Willebrand factor-cleaving protease ADAMTS13 is central to the pathophysiology of thrombotic thrombocytopenic purpura (TTP), a microangiopathic syndrome that presents as an acute medical emergency. In this review we will explore the evidence of a two-way relationship between TTP and ACS. Moreover, we will review the evidence emerged from epidemiological studies of an inverse relationship between the plasma levels of ADAMTS13 and the risk of ACS. METHODS AND RESULTS: Pubmed, MEDLINE and EMBASE, CINHAL, COCHRANE and Google Scholar databases were searched from inception to January 2017...
May 12, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28515627/frequently-asked-questions-on-seven-rare-adverse-events-following-immunization
#2
REVIEW
G L D'alò, E Zorzoli, A Capanna, G Gervasi, E Terracciano, L Zaratti, E Franco
Routine mass immunization programs have contributed greatly to the control of infectious diseases and to the improvement of the health of populations. Over the last decades, the rise of antivaccination movements has threatened the advances made in this field to the point that vaccination coverage rates have decreased and outbreaks of vaccine-preventable diseases have resurfaced. One of the critical points of the immunization debate revolves around the level of risk attributable to vaccination, namely the possibility of experiencing serious and possibly irreversible adverse events...
March 2017: Journal of Preventive Medicine and Hygiene
https://www.readbyqxmd.com/read/28515608/thrombotic-thrombocytopenic-purpura-in-a-case-of-dengue-fever-a-rare-presentation
#3
Abhijit S Gavali, Jayant Shelgaonkar, Sandip Bartakke
Here, we present an unusual occurrence of thrombotic thrombocytopenic purpura (TTP) in a case of dengue fever. Both the conditions are fatal and can result in significant mortality and morbidity if left untreated. In this case, as soon as, we diagnosed the patient as having TTP, we treated her with plasma exchange therapy, steroids, and monoclonal antibodies such as rituximab. The patient responded very well to the treatment and completely recovered from neurological symptoms and laboratory parameters also normalized...
April 2017: Indian Journal of Critical Care Medicine
https://www.readbyqxmd.com/read/28508977/thrombotic-microangiopathy-caused-by-interferon-%C3%AE-1b-for-multiple-sclerosis-a-case-report
#4
Haruomi Nishio, Tatsuo Tsukamoto, Takeshi Matsubara, Yoichiro Okada, Ryosuke Takahashi, Motoko Yanagita
A 41-year-old man with a history of multiple sclerosis (MS) developed thrombotic microangiopathy after taking interferon β-1b for 10 years. Although the relapse of his MS was well controlled under normal blood pressure, he had persistent nausea, anorexia, gait disturbance and visual disorder 1 month before admission. He showed lethargy and high blood pressure (180/102 mmHg). Laboratory test results revealed anemia and thrombocytopenia, elevated LDH and renal dysfunction. Urinary dipstick showed a 2+ result for proteinuria and 3+ for hematuria...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28500622/implementation-of-a-rapid-assay-of-adamts13-activity-was-associated-with-improved-30-day-survival-rate-in-patients-with-acquired-primary-thrombotic-thrombocytopenic-purpura-who-received-platelet-transfusions
#5
Yumi Yoshii, Yoshihiro Fujimura, Charles L Bennett, Ayami Isonishi, Norio Kurumatani, Masanori Matsumoto
BACKGROUND: Platelet (PLT) transfusions are probably harmful in patients with acquired idiopathic thrombotic thrombocytopenic purpura (aTTP). Introduction of a rapid assay for ADAMTS13 activity should reduce the time to definite diagnosis of aTTP, reduce the amount of inappropriately transfused PLT concentrates, and improve mortality and morbidity. STUDY DESIGN AND METHODS: We selected 265 aTTP patients with severe ADAMTS13 deficiency. Of these, 91 patients were diagnosed by March 2005 (Period 1), when ADAMTS13 activity was measured by von Willebrand factor multimer assay, which took 4 to 7 days until the result was reported...
May 12, 2017: Transfusion
https://www.readbyqxmd.com/read/28499011/fatal-case-of-chikungunya-and-concomitant-thrombotic-thrombocytopenic-purpura-in-french-guiana-during-air-flight-medical-evacuation
#6
Loïc Epelboin, Bastien Bidaud, Emilie Mosnier, Paul Le Turnier, Guillaume Vesin, Gaëlle Walter, Philippe Abboud, Stéphanie Houcke, Maïa Forgues, Gérald Egmann, Alain Stepanian, Félix Djossou
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy associated to severe ADAMTS13 deficiency. It has been linked to various viral infections. Among arboviruses, only Crimean-Congo haemorrhagic fever and dengue fever have been linked to this severe disease. We report the first documented case of TTP concomitant to Chikungunya virus infection.
September 1, 2017: Journal of Travel Medicine
https://www.readbyqxmd.com/read/28496360/epidural-anesthesia-for-labor-and-delivery-in-a-patient-with-may-hegglin-anomaly-a-case-report
#7
Annas Muhammad Muzannar, Mohammed Al Harbi, Raza Rathore, Nasser Tawfeeq, Freddie Wambi, Nasir Mahmood, Sonia Albrechtova, Vassilios Dimitriou
We report a case of May-Hegglin anomaly (MHA) in a woman who had a successful labor and delivery under epidural anesthesia. MHA is an inherited thrombocytopenia easily misdiagnosed as idiopathic (immune) thrombocytopenic purpura (ITP). Early and appropriate diagnosis of MHA during pregnancy is essential for optimal maternal and neonatal delivery outcome. Additionally, it can avoid unnecessary diagnostic studies, such as bone marrow aspiration and biopsy, and even harmful therapies with corticosteroids, immunosuppressive agents, and splenectomy...
2017: Local and Regional Anesthesia
https://www.readbyqxmd.com/read/28491169/submucosal-esophageal-hematoma-precipitated-by-chronic-idiopathic-thrombocytopenic-purpura
#8
Kanika Sharma, Yongdong Wang
Submucosal esophageal hematoma is an uncommon clinical entity. It can occur spontaneously or secondary to trauma, toxins, medical intervention, and in this case, coagulopathy. Management of SEH is supportive and aimed at its underlying cause. This article reports an 81-year-old male patient with chronic idiopathic thrombocytopenic purpura and hypertension that develops a submucosal esophageal hematoma.
June 2017: Radiology case reports
https://www.readbyqxmd.com/read/28484152/consensus-report-on-the-diagnosis-and-management-of-thrombotic-thrombocytopenic-purpura-2017
#9
Masanori Matsumoto
No abstract text is available yet for this article.
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28481690/two-year-antibody-persistence-in-children-vaccinated-at-12-15%C3%A2-months-with-a-measles-mumps-rubella-virus-vaccine-without-human-serum-albumin
#10
Andrea A Berry, Remon Abu-Elyazeed, Clemente Diaz-Perez, Maurice A Mufson, Christopher J Harrison, Michael Leonardi, Jerry D Twiggs, Christopher Peltier, Stanley Grogg, Antonio Carbayo, Steven Shapiro, Michael Povey, Carmen Baccarini, Bruce L Innis, Ouzama Henry
One combined measles-mumps-rubella (MMR) vaccine without Human Serum Albumin (HSA) is currently licensed in the USA (M-M-R II; Merck, USA) and another has been developed (Priorix™ [MMR-RIT, GSK, Belgium]). In this follow-up study, children from USA or Puerto Rico, who had received one dose of M-M-R II or MMR-RIT at 12-15 months of age in the primary study (NCT00861744), were followed-up for two years post-vaccination. Anti-measles and anti-rubella antibodies were measured using Enzyme-Linked Immunosorbent Assay (ELISA), and anti-mumps antibodies using ELISA and plaque reduction neutralization (PRN) assays...
May 8, 2017: Human Vaccines & Immunotherapeutics
https://www.readbyqxmd.com/read/28480350/therapeutic-efficacy-of-the-platelet-glycoprotein-ib-antagonist-anfibatide-in-murine-models-of-thrombotic-thrombocytopenic-purpura
#11
Liang Zheng, Yingying Mao, Mohammad S Abdelgawwad, Nicole K Kocher, Mandy Li, Xiangrong Dai, Benjamin Li, X Long Zheng
Thrombotic thrombocytopenic purpura (TTP), a potentially fatal blood clot disorder, is primarily caused by severe deficiency of plasma ADAMTS13 activity resulting from acquired autoantibodies. Plasma exchange is the only effective initial therapy. However, the high mortality rate and the complications associated with plasma exchange therapy remain a major concern. To address unmet clinical needs, therapeutic efficacies of anfibatide, a snake venom-derived platelet glycoprotein Ib antagonist, in murine models of spontaneous thrombocytopenia and shigatoxin-induced TTP were determined...
November 29, 2016: Blood Advances
https://www.readbyqxmd.com/read/28473932/acquired-thrombotic-thrombocytopenic-purpura-in-a-patient-with-pernicious-anemia
#12
Ramesh Kumar Pandey, Sumit Dahal, Kamal Fadlalla El Jack Fadlalla, Shambhu Bhagat, Bikash Bhattarai
Introduction. Acquired thrombotic thrombocytopenic purpura (TTP) has been associated with different autoimmune disorders. However, its association with pernicious anemia is rarely reported. Case Report. A 46-year-old male presented with blood in sputum and urine for one day. The vitals were stable. The physical examination was significant for icterus. Lab tests' results revealed leukocytosis, macrocytic anemia, severe thrombocytopenia, renal dysfunction, and unconjugated hyperbilirubinemia. He had an elevated LDH, low haptoglobin levels with many schistocytes, nucleated RBCs, and reticulocytes on peripheral smear...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28461837/a-case-of-sweet-s-syndrome-secondary-to-myelodysplastic-syndrome-diagnostic-and-treatment-challenges
#13
Sfrijan Doinita, Visan Simina-Maria, Diaconu Bianca, Zurac Sabina, Scurtu Cristian
Sweet's Syndrome also knows as acute febrile neutrophilic dermatosis, is a rare skin's condition, that can occur either idiopathic or secondary. In the case of the latter, the syndrome can develop after certain malignancies (paraneoplastic syndrome), because of exposure to some medication or post infectious. It is more frequent in women aged between 30 and 50 years, but concerning children, the disorder is extremely rare (8% of the total number of cases), having equal sex ratio distribution. We present the case of an 11 year old male, diagnosed with systemic form of SS associated with Myelodysplastic Syndrome...
June 2016: Mædica
https://www.readbyqxmd.com/read/28455885/an-extremely-rare-splice-site-mutation-in-the-gene-encoding-complement-factor-i-in-a-patient-with-atypical-hemolytic-uremic-syndrome
#14
Tina S Ipe, Jooeun Lim, Meredith Anne Reyes, Mike Ero, Christopher Leveque, Bradley Lewis, Jamey Kain
BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney failure. The disease is difficult to diagnose due to its similarity with other hematologic disorders, such as thrombotic thrombocytopenic purpura (TTP). However, genetic mutations are found in 50-70% of patients with aHUS and can be useful in its diagnosis. STUDY DESIGN AND METHODS: A 40-year-old male presented to our hospital with acute kidney injury, evidenced by high creatinine levels (8...
April 28, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28451010/-acquired-amegacaryocytic-thrombocytopenic-purpura-hiding-acute-myeloid-leukemia
#15
Hicham Eddou, Ali Zinebi, Abdelaziz Khalloufi, Mohammed Sina, Mehdi Mahtat, Kamal Doghmi, Mohammed Mikdame, Mohammed Karim Moudden, Mohammed El Baaj
Acquired amegakaryocytic thrombocytopenic purpura is a very rare condition characterized by severe thrombocytopenia linked to the reduction or disappearance of megakaryocytes in the bone marrow. It may be primary idiopathic or secondary to many pathological conditions including hematologic disorders. We report the case of a 24-year-old patient admitted for haemorrhagic syndrome caused by immunological thrombocytopenic purpura. The diagnosis was acquired amegakaryocytosis after the failure of corticotherapy and the performance of myelography...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28447417/diagnostic-approach-to-microangiopathic-hemolytic-disorders
#16
REVIEW
K Kottke-Marchant
Thrombotic micro-angiopathies (TMA) are a group of related disorders that are characterized by thrombosis of the microvasculature and associated organ dysfunction, and encompass congenital, acquired, and infectious etiologies. A hall mark of TMAs is the fragmentation of erythrocytes by the microvascular thrombi, resulting in a hemolytic anemia. There are several distinct pathophysiologies leading to microangiopathic hemolysis, ranging from decreased degradation of von Willebrand factor as seen in thrombotic thrombocytopenic purpura (TTP) to endothelial damage facilitated by Escherichia coli shiga toxin or complement dysregulation, seen in shiga toxin-related hemolytic-uremic syndrome (Stx-HUS) and complement-mediated TMA (also called atypical hemolytic-uremic syndrome), respectively...
May 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28445600/caplacizumab-reduces-the-frequency-of-major-thromboembolic-events-exacerbations-and-death-in-patients-with-acquired-thrombotic-thrombocytopenic-purpura
#17
F Peyvandi, M Scully, J A Kremer Hovinga, P Knöbl, S Cataland, K De Beuf, F Callewaert, H De Winter, R K Zeldin
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (aTTP) is a life-threatening autoimmune thrombotic microangiopathy. In spite of treatment with plasma exchange and immunosuppression, patients remain at risk for thrombotic complications, exacerbations and death. In the Phase II TITAN study, treatment with caplacizumab, an anti-vWF Nanobody(®) , was shown to reduce the time to confirmed platelet count normalization and exacerbations during treatment. OBJECTIVE: The clinical benefit of caplacizumab was further investigated in a post-hoc analysis of the incidence of major thromboembolic events and exacerbations during the study drug treatment period and TTP-related death during the study...
April 26, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28443948/relapse-of-congenital-thrombotic-thrombocytopenic-purpura-after-spontaneous-remission-in-a-second-trimester-primigravida-case-report-and-review-of-the-literature
#18
Donavan de Souza Lúcio, Jacqueline Foelkel Pignatari, Marcelo Gil Cliquet, Henri Augusto Korkes
CONTEXT: Thrombotic microangiopathy syndrome or thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) describes distinct diseases sharing common pathological features: microangiopathic hemolytic anemia and thrombocytopenia, without any other apparent cause. CASE REPORT: An 18-year-old second-trimester primigravida presented with a history of fifteen days of intense weakness, followed by diarrhea over the past six days. She reported having had low platelets since childhood, but said that she had never had bleeding or menstrual abnormalities...
April 20, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/28442528/inflammatory-bowel-disease-and-immune-thrombocytopenic-purpura-combined-immune-dysregulation-in-an-adolescent
#19
Karen Queliza, Faith D Ihekweazu, Arshaan Ali, Richard Kellermayer
Concomitant inflammatory bowel disease (IBD) and immune thrombocytopenic purpura (ITP) is a rare phenomenon. A shared immunologic pathway leading to mucosal inflammation and platelet destruction has been proposed. We report a case of a 14-year-old male who presented with abdominal pain, hematochezia, weight loss, and thrombocytopenia. Endoscopic and hematologic evaluations led to the diagnosis of ulcerative colitis (UC) and ITP, respectively. Initial treatment of his UC resulted in improvement in both gastrointestinal symptoms and platelet count...
March 2017: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/28442312/expert-statements-on-the-standard-of-care-in-critically-ill-adult-patients-with-atypical-haemolytic-uraemic-syndrome
#20
REVIEW
Elie Azoulay, Paul Knoebl, José Garnacho-Montero, Katerina Rusinova, Gennadii Galstian, Philippe Eggimann, Fekri Abroug, Dominique Benoit, Michael von Bergwelt-Baildon, Julia Wendon, Marie Scully
BACKGROUND: Atypical haemolytic uraemic syndrome (aHUS) presents similarly to thrombotic thrombocytopenic purpura (TTP), and other causes or conditions with thrombotic microangiopathy (TMA) such as DIC or sepsis. Similarity in clinical presentation may hinder diagnosis and optimal treatment selection in the urgent setting in the ICU. However, there is currently no consensus on the diagnosis or treatment of aHUS for ICU specialists. This review aims to summarise available data on the diagnosis and treatment strategies of aHUS in the ICU in order to enhance the understanding of aHUS diagnosis and outcomes in patients managed in the ICU...
April 22, 2017: Chest
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