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acquired hemophilia

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https://www.readbyqxmd.com/read/29144625/acquired-hemophilia-presenting-as-gross-hematuria-following-kidney-stone-a-case-report-and-review-of-the-literature
#1
Max Schmidt-Bowman, Lael Reinstatler, Eric P Raffin, Joseph E Yared, John D Seigne, Einar F Sverrisson
A rare condition in itself, acquired hemophilia A, seldom presents as isolated gross hematuria. It is a serious condition with a high mortality rate and thus clinical suspicion followed by prompt diagnosis is imperative (1). In fact, only 8 cases of such presentation of this condition have been reported thus far in the literature. Of these, none describe the initial presentation of hematuria with the inciting event of a kidney stone. We present a case of a 67-year-old man with signs and symptoms of nephrolithiasis accompanied by profuse hematuria, who was subsequently found to have developed expression of factor VIII inhibitor leading to acquired hemophilia A...
November 19, 2017: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/29126301/coagulation-testing-in-the-core-laboratory
#2
William E Winter, Sherri D Flax, Neil S Harris
Primary hemostasis begins with endothelial injury. VWF, produced by endothelial cells, binds to platelets and links them to subendothelial collagen. Platelet-derived ADP and thromboxane activate non-adhered platelets via their GPIIb/IIIa receptors, allowing these platelets to participate in platelet aggregation. Secondary hemostasis is initiated with the binding of factor VII to extravascular tissue factor (TF). Factors II, VII, IX and X are vitamin K-dependent factors. The role of vitamin K is to assist in the addition of gamma carboxylate groups to glutamic acids in the "GLA" domains of these factors...
November 8, 2017: Laboratory Medicine
https://www.readbyqxmd.com/read/29112188/diagnosis-and-treatment-options-of-acquired-hemophilia-a-single-center-experience
#3
Bożena Sokołowska, Justyna Kozińska, Magdalena Kozioł, Ewa Wąsik-Szczepanek, Dariusz Szczepanek, Marek Hus
No abstract text is available yet for this article.
November 6, 2017: Polish Archives of Internal Medicine
https://www.readbyqxmd.com/read/29099367/dental-management-of-patients-with-inherited-bleeding-disorders-a-multidisciplinary-approach
#4
Hassan Abed, Abdalrahman Ainousa
Bleeding disorders can be inherited or acquired and demonstrate different levels of severity. Dentists may be called on to treat patients who have bleeding disorders such as hemophilia A and von Willebrand disease (vWD). Dental extraction in any patient with clotting factor defects can result in a delayed bleeding episode. Local hemostatic measures provide effective results in a majority of cases but are insufficient in patients with severe hemophilia A and vWD. Therefore, consultation with the patient's hematologist is required to ensure preoperative prophylactic coverage...
November 2017: General Dentistry
https://www.readbyqxmd.com/read/29095762/acquired-hemophilia-with-thrombosis-in-a-cancer-patient-an-unusual-presentation
#5
Senem Maral, Sule Mine Bakanay, Imdat Dilek
: Acquired hemophilia A (AHA) which presents with spontaneous severe intramuscular, mucosal and/or subcutaneous bleeding is a rare bleeding disorder. Even 50% of AHA patients are defined as idiopathic; 10% of cases are related with malignancy. Here, we present a case of AHA in a 43-year-old lady who was diagnosed with malignancy and venous thromboembolism on vena cava 2 years ago. To the best of our knowledge, this is the first report in literature presented with both acquired hemophilia and thrombosis associated with malignancy...
November 1, 2017: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29078851/acquired-hemophilia-and-anti-hu-paraneoplastic-neurologic-syndrome-in-small-cell-lung-cancer
#6
Efi Aggelopoulou, Gerasimos Evangelatos, Konstantina Tzavida, Vasiliki Koulouri, Nikolaos Lazaridis
No abstract text is available yet for this article.
October 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/29027258/acquired-factor-xi-deficiency-and-therapeutic-plasma-exchange
#7
Geoffrey D Wool, Angela Treml, Jonathan L Miller
Congenital factor XI (FXI) deficiency is associated with a variable bleeding phenotype. Recent reports have documented the use of therapeutic plasma exchange to rapidly and isovolumetrically increase FXI levels before invasive procedures in patients with congenital FXI deficiency. We report a case of acquired FXI deficiency in a pregnant woman with lupus. We proved that the inhibitor was an IgG, therefore potentially capable of crossing the placenta. While immune suppression eliminated detectable circulating inhibitor, the woman's FXI remained quite low...
October 13, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28960371/acquired-hemophilia-a-resolution-in-an-hcv-hiv-coinfected-patient-with-cure-of-hepatitis-c-by-direct-antiviral-agents
#8
Laetitia Mauge, Juliette Pavie, Dominique Batisse, Luc Darnige
No abstract text is available yet for this article.
September 28, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28919213/treatment-and-complications-in-acquired-hemophilia-a-experience-from-a-single-center
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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