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acquired hemophilia

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https://www.readbyqxmd.com/read/28919213/treatment-and-complications-in-acquired-hemophilia-a-experience-from-a-single-center
#1
Laura Entrena Ureña, Dolores Fernández Jiménez, María José Gutiérrez Pimentel
No abstract text is available yet for this article.
September 14, 2017: Medicina Clínica
https://www.readbyqxmd.com/read/28903884/successful-outcome-of-severe-intra-cerebral-bleeding-associated-with-acquired-factor-v-inhibition-utilization-of-multiple-therapeutic-agents
#2
Panagiotis Andreadis, Katerina Kafantari, Aleka Agapidou, Sofia Vakalopoulou, Efthymia Vlachaki
BACKGROUND: Acquired coagulation Factor inhibitors are antibodies that either inhibit the activity or increase the clearance of a clotting factor leading to an increased risk of bleeding. Most of the times, the disorder is attributed to Factor VIII inhibition (Acquired Hemophilia A), however other coagulation factors could also be implicated. CASE REPORT: We herein would like to report an interesting case of a patient who underwent Coronary Artery Bypass Graft and received antibiotic treatment after surgery with a third-generation cephalosporin...
September 13, 2017: Balkan Medical Journal
https://www.readbyqxmd.com/read/28886353/initial-manifestation-of-acquired-hemophilia-a-after-a-routine-tooth-extraction-a-case-report-and-literature-review
#3
Nicholas A Bennetts, James E Mergelmeyer, Eric J Reimer, James C Melville
Although surgical treatment of patients on anticoagulation regimens is common practice among oral and maxillofacial surgeons, unexpected and unknown coagulopathies can have devastating and catastrophic consequences for the most routine of procedures. Acquired hemophilia A (AHA) is an extremely rare life-threatening bleeding disorder characterized by autoantibodies directed against circulating coagulation factor VIII. The effects of AHA can produce catastrophic bleeding and hematomas. The effect of this uncontrolled hemorrhage after dentoalveolar surgery can mimic severe head and neck infection by causing dysphagia, odynophagia, and acute airway complications...
August 12, 2017: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28870236/acquired-hemophilia-a-in-an-advanced-age-patient-of-hispanic-origin-a-case-report
#4
Nalyssa I Rivera Cora, Freddie Irizarry Delgado, Santa M Merle Ramírez, Jorge Vera Quiñones
BACKGROUND: Acquired Hemophilia A (AHA) is a rare hematological disorder that exhibits an incidence of approximately 1.5 cases per million patients a year. It is characterized by the development of autoantibodies against circulating Factor VIII coagulation proteins which, in turn, which in turn lead to potentially life-threatening hemorrhagic episodes. The incidence of AHA increases with age; with 80% of the affected patient population encompassing men and women that are 65 years or older...
September 4, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/28830606/thrombin-generation-assays-for-global-evaluation-of-the-hemostatic-system-perspectives-and-limitations
#5
Rita Carolina Figueiredo Duarte, Cláudia Natália Ferreira, Danyelle Romana Alves Rios, Helton José Dos Reis, Maria das Graças Carvalho
The existing techniques to evaluate hemostasis in clinical laboratories are not sensitive enough to detect hypercoagulable and mild hypocoagulable states. Under different experimental conditions, the thrombin generation test may meet these requirements. This technique evaluates the overall balance between procoagulant and anticoagulant forces and has provided new insights in our understanding of the coagulation cascade, as well as of the diagnosis of hypocoagulability and hypercoagulability conditions. Thrombin generated in the thrombin generation test can be quantified as platelet-rich or platelet-poor plasma using the calibrated automated thrombogram method, which monitors the cleavage of a fluorogenic substrate that is simultaneously compared to the known thrombin activity in a non-clotting plasma sample...
July 2017: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/28804837/detection-and-measurement-of-factor-inhibitors
#6
Geoffrey Kershaw
Specific coagulation factor inhibitors, most notably to coagulation factor VIII, can develop in patients with hemophilia after exposure to replacement factor or as an acquired (autoimmune) event. Such inhibitors can be detected by a combination of prolonged coagulation screening tests, such as the activated partial thromboplastin time, plus a non-correcting mixing test. Non-correction may only be evident, or else may be extenuated, with incubation at 37 °C. The Bethesda assay for quantitation of inhibitor strength is based on a combination of mixing tests followed by specific factor assays...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28789965/application-of-polyploid-adeno-associated-virus-vectors-for-transduction-enhancement-and-neutralizing-antibody-evasion
#7
Zheng Chai, Junjiang Sun, Kelly Michelle Rigsbee, Mei Wang, R Jude Samulski, Chengwen Li
Adeno-associated virus (AAV) vectors have been used successfully in clinical trials for patients with hemophilia or blindness, but pre-existing neutralizing antibodies (Nab) are common in the general population and exclude many patients from clinical trials. Exploration of effective strategies to enhance AAV transduction and escape from Nab activity is still imperative. Previous studies have shown the compatibility of capsids from AAV serotypes and homology of recognition sites of AAV Nab located on different capsid subunits from one virion...
August 5, 2017: Journal of Controlled Release: Official Journal of the Controlled Release Society
https://www.readbyqxmd.com/read/28769599/identification-of-people-with-acquired-hemophilia-in-a-large-electronic-health-record-database
#8
Michael Wang, Anissa Cyhaniuk, David L Cooper, Neeraj N Iyer
BACKGROUND: Electronic health records (EHRs) can provide insights into diagnoses, treatment patterns, and clinical outcomes. Acquired hemophilia (AH) is an ultrarare bleeding disorder characterized by factor VIII inhibiting autoantibodies. AIM: To identify patients with AH using an EHR database. METHODS: Records were accessed from a large EHR database (Humedica) between January 1, 2007 and July 31, 2013. Broad selection criteria were applied using the International Classification of Diseases, Ninth Revision, clinical modification (ICD-9-CM) code for intrinsic circulating anticoagulants (286...
2017: Journal of Blood Medicine
https://www.readbyqxmd.com/read/28767473/acquired-hemophilia-a-after-hepatic-yttrium-90-radioembolization-a-case-report
#9
Susanna Tribuzi, Alessia Naccarato, Lorella Pelagalli, Marco Covotta, Giulia Torregiani, Claudia Claroni, Ester Forastiere
Acquired hemophilia is a rare but potentially life-threatening bleeding disorder caused by the development of autoantibodies (inhibitors) directed against plasma coagulation factors, most frequently factor VIII. We report a case of a 65-year-old man with hepatocellular carcinoma who bled massively after a hepatic Yttrium-90 radioembolization procedure (Selective Internal Radiation Therapy with Yttrium-90 Resin Microspheres [SIRTex]). An acquired deficiency of factor VIII was diagnosed and successfully treated with recombinant activated factor VII and immunosuppression...
August 1, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28738402/biodistribution-of-liver-derived-mesenchymal-stem-cells-after-peripheral-injection-in-a-hemophilia-a-patient
#10
Etienne M Sokal, Catherine Anne Lombard, Véronique Roelants, Mustapha Najimi, Sharat Varma, Camillo Sargiacomo, Joachim Ravau, Giuseppe Mazza, François Jamar, Julia Versavau, Vanessa Jacobs, Marc Jacquemin, Stéphane Eeckhoudt, Catherine Lambert, Xavier Stéphenne, Françoise Smets, Cédric Hermans
BACKGROUND: With the exception of liver transplantation, there is no cure for hemophilia, which is currently managed by preemptive replacement therapy. Liver-derived stem cells are in clinical development for inborn and acquired liver diseases and could represent a curative treatment for hemophilia A. The liver is a major factor VIII (FVIII) synthesis site, and mesenchymal stem cells have been shown to control joint bleeding in animal models of hemophilia. Adult-derived human liver stem cells (ADHLSCs) have mesenchymal characteristics and have been shown able to engraft in and repopulate both animal and human livers...
August 2017: Transplantation
https://www.readbyqxmd.com/read/28680617/a-case-of-severe-unprovoked-hemorrhage-in-an-elderly-male-a-case-report
#11
Petros Ioannou, Emmanouela Tsagkaraki, Constantinos Tsioutis, Maria Devetzoglou, Irene Xylouri, Achilleas Gikas, Symeon Panagiotakis
Acquired hemophilia is a rare but potentially fatal clinical condition requiring clinical suspicion to reach to a diagnosis, especially in elder patients. This diagnosis should be suspected in patients that present with unexplained persistent bleeding from skin, soft tissues, and mucosa and have a prolonged aPTT.
July 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28670081/treatment-of-chronic-lymphocytic-leukemia-small-lymphocytic-lymphoma-presenting-simultaneously-with-acquired-hemophilia-and-warm-autoimmune-hemolytic-anemia
#12
Chelsea Williams, Christian Cable, Julia Choi
Since both acquired factor VIII inhibitor in non-hemophiliac patients and warm autoimmune hemolytic anemia are uncommon disorders with no case-controlled trials, managing these diseases can be challenging. We present a case of a 75-year-old man in whom both diseases were present simultaneously with life-threatening bleeding. This case is an example of the successful initial management and long-term treatment of acquired hemophilia A, warm autoimmune hemolytic anemia, and chronic lymphocytic leukemia/small lymphocytic lymphoma with rituximab, prednisone, and cyclophosphamide...
July 2017: Proceedings of the Baylor University Medical Center
https://www.readbyqxmd.com/read/28648539/prothrombin-complex-concentrates-in-pediatric-cardiac-surgery-the-current-state-and-the-future
#13
REVIEW
Elena Ashikhmina, Sameh Said, Mark M Smith, Vilmarie Rodriguez, William C Oliver, Gregory A Nuttall, Joseph A Dearani, Hartzell V Schaff
BACKGROUND: After decades of practice of pediatric cardiac surgery, postoperative bleeding due to the immaturity of hemostasis, hemodilution, and hypothermia remains a concern. Recently, a new approach for adult coagulopathy after bypass has emerged. Prothrombin complex concentrates (PCCs), designed to treat bleeding in hemophilia patients, are safely and efficiently used off label for hemorrhage after bypass. However, optimal dosing, indications and contraindications, and laboratory tests to assess the efficacy of PCC use in children have not yet been established...
June 22, 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28597369/first-report-of-real-time-monitoring-of-coagulation-function-potential-and-igg-subtype-of-anti-fviii-autoantibodies-in-a-child-with-acquired-hemophilia-a-associated-with-streptococcal-infection-and-amoxicillin
#14
Masahiro Takeyama, Keiji Nogami, Takahiro Kajimoto, Kenichi Ogiwara, Tomoko Matsumoto, Midori Shima
We describe an 8-year-old boy with acquired hemophilia A (AHA) associated with streptococcal infection and amoxicillin. Laboratory data revealed low factor VIII activity (FVIII:C, 1.5 IU/dl), and FVIII inhibitor (15.9 BU/ml). Comprehensive coagulation function assays, including rotation thromboelastometry (ROTEM(®)), revealed a markedly prolonged clotting time. Thrombin and plasmin generation (TG/PG) appeared to be moderately impaired. The inhibitor epitope of his anti-FVIII autoantibody recognized light and heavy chains...
June 8, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28567535/immune-mediated-syndromes-following-intravenous-bisphosphonate-therapy
#15
Noa Markovits, Ronen Loebstein, Ilan Bank
OBJECTIVES: Intravenous (IV) infusion of aminobisphosphonates (ABP) induces cytokine release by peripheral blood Vγ9δ2 T cells, resulting in an immediate short-term inflammatory response in up to 50% of patients. We evaluated possible long-term pro-inflammatory effects of IV ABP. METHODS: Retrospective case-series study from one rheumatology specialist's clinic. 2261 electronic charts were reviewed for administration of 'zoledronate' or different brand names of zoledronic acid, and relevant clinical data was retrieved for patients who had received the infusion...
May 31, 2017: Inflammopharmacology
https://www.readbyqxmd.com/read/28565741/-analysis-of-clinical-efficacy-of-recombinant-activated-factor-%C3%A2-on-bleeding-in-patients-with-hematologic-disorders
#16
W Liu, F Xue, X F Liu, E L Jiang, D L Yang, K Q Liu, Z J Xiao, F K Zhang, S Z Feng, M Z Han, L Zhang, R C Yang
Objective: To investigate the treatment efficacy of recombinant activated factor Ⅶ (rFⅦa) for bleeding among patients with hematologic disorders. Methods: A total of 38 times of bleeding in 31 patients with hematological disease treated with rFⅦa were analyzed retrospectively. Results: The clinical effective rate of rFⅦa for bleeding management in acquired hemophilia A (AHA) patients/hemophilia patients with inhibitor, acute promyelocytic leukemia (APL) patients and patients with non-APL leukemia was 90% (9/10) , 71...
May 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28523993/acquired-hemophilia-as-initial-presentation-in-a-patient-with-systemic-lupus-erythematosus
#17
Zohre Khodamoradi, Mohammad Ali Nazarinia, Somaye Bazdar
BACKGROUND: Acquired hemophilia is a rare bleeding disease but may be associated with some autoimmune diseases. Acquired hemophilia may be the result of autoantibodies against factor VIII. CASE PRESENTATION: In this study, we describe a 55 year old patient who developed hematoma and hematuria due to acquired hemophilia secondary to systemic lupus erythematosus(SLE). Then, she developed arthritis and thrombosis after some evaluation. Laboratory data showed prolonged aPTT, normal PT and platelet, low FVIII, high FVIII inhibitor, ANA, anti-ds-DNA and anti-cardiolipin...
May 19, 2017: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/28507083/frequency-and-epitope-specificity-of-anti-factor-viii-c1-domain-antibodies-in-acquired-and-congenital-hemophilia-a
#18
Joerg Kahle, Aleksander Orlowski, Diana Stichel, John F Healey, Ernest T Parker, Marc Jacquemin, Manuela Krause, Andreas Tiede, Dirk Schwabe, Pete Lollar, Christoph Königs
Several studies showed that neutralizing anti-factor VIII (anti-fVIII) antibodies (inhibitors) in patients with acquired hemophilia A (AHA) and congenital hemophilia A (HA) are primarily directed to the A2 and C2 domains. In this study, the frequency and epitope specificity of anti-C1 antibodies were analyzed in acquired and congenital hemophilia inhibitor patients (n = 178). The domain specificity of antibodies was studied by homolog-scanning mutagenesis (HSM) with single human domain human/porcine fVIII proteins and antibody binding to human A2, C1, and C2 domains presented as human serum albumin (HSA) fusion proteins...
August 10, 2017: Blood
https://www.readbyqxmd.com/read/28470674/acquired-hemophilia-a-updated-review-of-evidence-and-treatment-guidance
#19
REVIEW
Rebecca Kruse-Jarres, Christine L Kempton, Francesco Baudo, Peter W Collins, Paul Knoebl, Cindy A Leissinger, Andreas Tiede, Craig M Kessler
Acquired hemophilia A (AHA) is a rare disease resulting from autoantibodies (inhibitors) against endogenous factor VIII (FVIII) that leads to bleeding, which is often spontaneous and severe. AHA tends to occur in elderly patients with comorbidities and is associated with high mortality risk from underlying comorbidities, bleeding, or treatment complications. Treatment, which consists of hemostatic management and eradication of the inhibitors, can be challenging to manage. Few data are available to guide the management of AHA-related bleeding and eradication of the disease-causing antibodies...
July 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28458935/successful-management-of-acquired-hemophilia-a-associated-with-bullous-pemphigoid-a-case-report-and-review-of-the-literature
#20
Quentin Binet, Catherine Lambert, Laurine Sacré, Stéphane Eeckhoudt, Cedric Hermans
Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening. Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association...
2017: Case Reports in Hematology
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