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https://www.readbyqxmd.com/read/28447417/diagnostic-approach-to-microangiopathic-hemolytic-disorders
#1
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
#2
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
#3
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
#4
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
#5
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
https://www.readbyqxmd.com/read/28440561/anterior-st-elevation-myocardial-infarction-induced-by-rituximab-infusion-a-case-report-and-review-of-the-literature
#6
K Sharif, A Watad, N L Bragazzi, E Asher, A Abu Much, Y Horowitz, M Lidar, Y Shoenfeld, H Amital
WHAT IS KNOWN AND OBJECTIVES: Rituximab is a chimeric monoclonal anti-CD20 antibody approved for the treatment of some lymphoid malignancies as well as for autoimmune diseases including rheumatoid arthritis (RA), idiopathic thrombocytopenic purpura (ITP) and vasculitis. Generally, rituximab is well tolerated; nevertheless, some patients develop adverse effects including infusion reactions. Albeit rare, these reactions may in some cases be life-threatening conditions. Rituximab cardiovascular side effects include more common effects such as hypertension, oedema and rare cases of arrhythmias and myocardial infarction...
June 2017: Journal of Clinical Pharmacy and Therapeutics
https://www.readbyqxmd.com/read/28424819/plasma-micrornas-characterising-patients-with-immune-thrombocytopenic-purpura
#7
Bin Zuo, Juping Zhai, Lifang You, Yunxiao Zhao, Jianfeng Yang, Zhen Weng, Lan Dai, Qingyu Wu, Changgeng Ruan, Yang He
Altered microRNA (miRNA) expression has been reported in patients with immune thrombocytopenic purpura (ITP). However, the detailed expression profiling of cell-free circulating miRNAs in ITP patients has not been fully investigated. In this study, we aimed to examine plasma miRNAs in ITP patients and evaluate their diagnostic values. Plasma samples from 74 ITP patients and 58 healthy controls were obtained and allocated into discovery, validation, and therapy-response sets. Initial screen with a miRNA microarray assay identified 23 miRNAs with different levels between ITP patients and healthy controls (>1...
April 20, 2017: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/28424750/celiac-disease-presenting-with-immune-thrombocytopenic-purpura
#8
Hakan Sarbay, Billur Cosan Sarbay, Mehmet Akın, Halil Kocamaz, Mahya Sultan Tosun
Celiac disease (CD) is an immunological disorder. Clinical manifestations occur as a result of intestinal mucosa damage and malabsorption. CD is also associated with extraintestinal manifestations and autoimmune disorders. The coexistence of CD and autoimmune diseases has been described before. In this article, a patient with CD presenting with thrombocytopenia is discussed.
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/28417064/adenocarcinoma-of-the-lung-presenting-as-thrombotic-thrombocytopenic-purpura
#9
Ahmed Dirweesh, Waqas Siddiqui, Muhammad Khan, Praneet Iyer, Marc Seelagy
Acquired thrombotic thrombocytopenic purpura (TTP) usually presents as severe microangiopathic hemolytic anemia (MAHA) and thrombocytopenia in a previously healthy individual. It occurs in approximately three in one million adults and 1 in 10 million children annually. The incidence is increased in females and blacks. Diagnosing TTP and initiating therapy with plasma exchange is a must to avoid preventable complications. Acquired thromotic microangiopathy has been linked to collagen vascular diseases, use of certain medications, organ transplants, infections, pregnancy and cancer...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28416509/none-of-the-above-thrombotic-microangiopathy-beyond-ttp-and-hus
#10
Camila Masias, Sumithira Vasu, Spero R Cataland
Acquired thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are appropriately at the top of a clinician's differential when a patient presents with a clinical picture consistent with an acute thrombotic microangiopathy (TMA). However, there are several additional diagnoses that should be considered in patients presenting with an acute TMA, especially in patients with non-deficient ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity (>10%)...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28416507/thrombotic-thrombocytopenic-purpura
#11
Bérangère S Joly, Paul Coppo, Agnes Veyradier
Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening thrombotic micro-angiopathy characterized by a microangiopathic hemolytic anemia, severe thrombocytopenia and organ ischemia linked to disseminated microvascular platelet rich-thrombi. TTP is specifically related to a severe deficiency in ADAMTS13 (A Disintegrin and Metalloprotease with ThromboSpondin type 1 repeats, member 13), the specific von Willebrand factor-cleaving pro-tease. ADAMTS13 deficiency is most frequently acquired via ADAMTS13 autoantibodies but rarely, it is inherited via mutations of ADAMTS13 gene...
April 17, 2017: Blood
https://www.readbyqxmd.com/read/28413179/cyclic-thrombocytopenia-synchronizing-with-the-menstrual-cycle-showing-periodic-phases-of-thrombocytopenia-and-rebound-thrombocytosis
#12
Taro Kurihara, Masahiko Sumi, Hiroko Kaiume, Wataru Takeda, Takehiko Kirihara, Keijiro Sato, Toshimitsu Ueki, Yuki Hiroshima, Hikaru Kobayashi
A 37-year-old woman was admitted to our hospital for purpura involving the extremities and thrombocytopenia. Prednisolone (PSL) was administered based on a diagnosis of idiopathic thrombocytopenic purpura (ITP), but was not effective for maintaining her platelet count within the normal range, which showed cyclic fluctuation corresponding to the menstrual cycle. Therefore, we discontinued PSL, and cyclic thrombocytopenia (CTP) was diagnosed. CTP is a rare disease which is usually treated as ITP but with no response...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28413093/-thrombotic-thrombocytopenia-purpura-in-martinique-retrospective-study-between-2008%C3%A2-and-2015
#13
M Patient, P Fuseau, C Deligny
INTRODUCTION: Some studies suggest that thrombotic thrombocytopenic purpura (TTP) occurs more often in African Americans. However there is low evidence for this in the literature. The aim of our study was to describe the clinical and biological characteristics of TTP in the Afro-Caribbean population of Martinique. METHODS: We retrospectively analysed all patients with TTP diagnosed at the Fort-de-France hospital between 2008, January 1st and 2015, December 31st...
April 13, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28411378/immune-responses-and-long-term-disease-recurrence-status-after-telomerase-based-dendritic-cell-immunotherapy-in-patients-with-acute-myeloid-leukemia
#14
Hanna J Khoury, Robert H Collins, William Blum, Patrick S Stiff, Laurence Elias, Jane S Lebkowski, Anita Reddy, Kevin P Nishimoto, Debasish Sen, Edward D Wirth, Casey C Case, John F DiPersio
BACKGROUND: Telomerase activity in leukemic blasts frequently is increased among patients with high-risk acute myeloid leukemia (AML). In the current study, the authors evaluated the feasibility, safety, immunogenicity, and therapeutic potential of human telomerase reverse transcriptase (hTERT)-expressing autologous dendritic cells (hTERT-DCs) in adult patients with AML. METHODS: hTERT-DCs were produced from patient-specific leukapheresis, electroporated with an mRNA-encoding hTERT and a lysosomal-targeting sequence, and cryopreserved...
April 14, 2017: Cancer
https://www.readbyqxmd.com/read/28408234/the-acute-hemolytic-anemias-the-importance-of-emergency-diagnosis-and-management
#15
Jennifer J Robertson, Elizabeth Brem, Alex Koyfman
BACKGROUND: Hemolytic anemias are defined by the premature destruction of red blood cells. These anemias have many causes that are mostly due to chronic diseases, but, occasionally, cases of acute life-threatening hemolysis can occur. OBJECTIVE: The objectives of this article were to review the pathophysiology of hemolytic anemias, to discuss the general emergency department (ED) evaluation, and to discuss the assessment and treatment of important and "cannot miss" hemolytic diseases...
April 10, 2017: Journal of Emergency Medicine
https://www.readbyqxmd.com/read/28405075/acquired-thrombotic-thrombocytopenic-purpura-and-atypical-hemolytic-uremic-syndrome-successfully-treated-with-eculizumab
#16
Appalanaidu Sasapu, Michele Cottler-Fox, Pooja Motwani
Acquired idiopathic thrombotic thrombocytopenic purpura is a life-threatening disease with a mortality of up to 90%, if not promptly recognized and treated. We report a 64-year-old woman with this condition who presented with left-sided weakness and seizure-like activity preceded by headache and easy bruising. She did not achieve optimal response to plasma exchange, corticosteroids, rituximab, and vincristine. We initiated treatment with eculizumab, following which she had durable remission that continued for 30 months after discontinuation of the drug...
April 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28401611/pegylated-bovine-carboxyhaemoglobin-utilisation-in-a-thrombotic-thrombocytopenic-purpura-patient
#17
C Sam, P Desai, D Laber, A Patel, N Visweshwar, M Jaglal
OBJECTIVE: To determine if pegylated bovine carboxyhaemoglobin can be utilised in a thrombotic thrombocytopenic purpura (TTP) patient. BACKGROUND: TTP is a condition characterized by thrombotic microangiopathy and has a high mortality rate when left untreated. Therapeutic plasma exchange is well established as the most effective and evidence-based treatment of TTP. METHODS: The ability to administer plasma exchange therapy is limited in Jehovah's Witnesses who decline blood products due to religious beliefs...
April 12, 2017: Transfusion Medicine
https://www.readbyqxmd.com/read/28395447/-quantity-of-treg-cells-and-th17-cells-in-spleen-of-primary-immune-thrombocytopenic-purpura-patients
#18
Y Y Yang, F Q Zhang, Y Pan, F Chen, M J Huang, D Y Zeng, H G Huang
Objective: To observe the quantity of Treg cells and Th17 cells in spleen of adult primary immune thrombocytopenic purpura (ITP) patients. Methods: 43 ITP cases with splenectomy treatment were enrolled from December 2008 to June 2016 at Union Hospital of Fujian Medical University, including 20 males and 23 females with a median age of 36 (18-76) years. The controls were thirty patients who underwent splenectomy because of pancreatic diseases or splenic impairment, including 21 males and 9 females with a median age of 47 (21-69) years...
March 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28387984/practical-issues-in-using-eculizumab-for-children-with-atypical-hemolytic-uremic-syndrome-in-the-acute-phase-a-review-of-4-patients
#19
Chikako Terano, Kenji Ishikura, Riku Hamada, Yasuhiro Yoshida, Wataru Kubota, Yusuke Okuda, Shunsuke Shinozuka, Ryoko Harada, Sunao Iyoda, Yoshihiro Fujimura, Yuko Hamasaki, Hiroshi Hataya, Masataka Honda
AIM: Recently eculizumab, a monoclonal antibody to C5, was found to improve the disease course of atypical hemolytic uremic syndrome (aHUS) and has been recommended as the first line treatment by an international consensus guideline. However, several practical issues in the use of eculizumab for the acute phase of aHUS have yet to be resolved. METHODS: Children who received eculizumab with diagnosis of aHUS between March 2010 and December 2015 at Tokyo Metropolitan Children's Medical Center were enrolled...
April 7, 2017: Nephrology
https://www.readbyqxmd.com/read/28383422/acquired-idiopathic-thrombotic-thrombocytopenic-purpura-successfully-treated-with-intravenous-immunoglobulin-and-glucocorticoid-a-case-report
#20
Hiro Nakao, Akira Ishiguro, Nahoko Ikoma, Kentaro Nishi, Chemin Su, Hisaya Nakadate, Mitsuru Kubota, Masaki Hayakawa, Masanori Matsumoto
RATIONALE: Plasma exchange is the principal treatment for acquired thrombotic thrombocytopenic purpura (TTP) but is invasive and may have adverse effects. Reports of immunoglobulin therapy for acquired TTP without plasma exchange are rare. PATIENT CONCERNS: A 14-year-old girl was admitted because of hemolytic anemia and thrombocytopenia. DIAGNOSIS: Acquired TTP was diagnosed based on low ADAMTS13 (a disintegrin-like and metalloproteinase with thrombospondin type 1 motif, 13) activity and a high ADAMTS13 inhibitor level...
April 2017: Medicine (Baltimore)
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