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Factor VIII inhibitor

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https://www.readbyqxmd.com/read/29777571/optimization-of-pre-analytical-heat-treatment-for-inhibitor-detection-in-haemophilia-a
#1
P Batty, D P Hart, S Platton
INTRODUCTION: Factor VIII (FVIII) antibody formation is the greatest clinical and laboratory challenge within the haemophilia centre. The Nijmegen-Bethesda assay (NBA) is the gold standard for inhibitor quantification, but affected by pre-analytical variables including a patient's FVIII activity (FVIII:C). Pre-analytical heat treatment (PHT) provides a methodology for inhibitor testing when measurable FVIII:C is present. METHODS: We evaluated the effect of different PHT conditions (time/temperature) on FVIII:C as well as on potency of inhibitory activity in samples containing FVIII:C (endogenous pooled plasma and exogenous recombinant FVIII (rFL-FVIII) concentrate) or FVIII inhibitor...
May 18, 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29769259/novel-therapeutics-for-hemophilia-and-other-bleeding-disorders
#2
Michael U Callaghan, Robert Sidonio, Steven W Pipe
Hemophilia and von Willebrand disease are the most common congenital bleeding disorders. Treatment for these disorders has focused on replacement of the missing coagulation factor to prevent or treat bleeding. New technologies and insights into hemostasis have driven the development of many promising new therapies for hemophilia and von Willebrand disease. Emerging bypass agents including zymogen-like factor IXa and Xa molecules are in development and a bispecific antibody, emicizumab, demonstrated efficacy in a phase III trial in people with hemophilia A and inhibitors...
May 16, 2018: Blood
https://www.readbyqxmd.com/read/29769125/observational-study-of-coagulation-activation-in-early-breast-cancer-development-of-a-prognostic-model-based-on-data-from-the-real-world-setting
#3
Chiara Mandoj, Laura Pizzuti, Domenico Sergi, Isabella Sperduti, Marco Mazzotta, Luigi Di Lauro, Antonella Amodio, Silvia Carpano, Anna Di Benedetto, Claudio Botti, Francesca Ferranti, Anna Antenucci, Maria Gabriella D'Alessandro, Paolo Marchetti, Silverio Tomao, Giuseppe Sanguineti, Antonio Giordano, Marcello Maugeri-Saccà, Gennaro Ciliberto, Laura Conti, Patrizia Vici, Maddalena Barba
BACKGROUND: Cancer and coagulation activation are tightly related. The extent to which factors related to both these pathologic conditions concur to patient prognosis intensely animates the inherent research areas. The study herein presented aimed to the development of a tool for the assessment and stratification of risk of death and disease recurrence in early breast cancer. METHODS: Between 2008 and 2010, two hundreds thirty-five (N: 235) patients diagnosed with stage I-IIA breast cancer were included...
May 16, 2018: Journal of Translational Medicine
https://www.readbyqxmd.com/read/29743396/-congenital-factor-v-and-factor-viii-deficiency-discovered-in-an-elderly-patient-with-abnormal-bleeding-after-trauma
#4
Yoshiyuki Ogawa, Kunio Yanagisawa, Yuri Uchiyama, Akira Matsumoto, Madoka Inoue, Kohtaro Toyama, Yuri Miyazawa, Naomichi Matsumoto, Hiroshi Handa
Congenital combined deficiency of coagulation factor V (FV) and factor VIII (FVIII) (F5F8D) is a rare autosomal recessive bleeding disorder caused by mutations in lectin mannose-binding type 1 (LMAN1) or multiple coagulation factor deficiency 2 (MCFD2) encoding chaperone molecules involved in the intracellular transport of FV and FVIII. Here, we report a case of F5F8D in an elderly patient diagnosed with hematoma after a right thigh injury. A 71-year-old male had a history of abnormal bleeding after tooth extraction and cholecystectomy...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29741261/new-challenges-and-best-practices-for-the-laboratory-monitoring-of-factor-viii-and-factor-ix-replacement
#5
REVIEW
D Van den Bossche, K Peerlinck, M Jacquemin
Several recombinant factor VIII and factor IX concentrates with extended half-life (EHL) have recently been validated by clinical studies. The availability of these novel concentrates is expected to significantly facilitate the treatment of patients with hemophilia A and B. However, the modification applied to these molecules has introduced variations in their activity measurement in routine coagulation assays. Depending on the assays, underestimations of up to 10-fold or overestimations of up to approximately 30-fold in the measurements of the recovery have been reported in some factor concentrates...
May 2018: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/29734520/routine-measurements-of-factor-viii-activity-and-inhibitor-titer-in-the-presence-of-emicizumab-utilizing-anti-idiotype-monoclonal-antibodies
#6
Keiji Nogami, Tetsuhiro Soeda, Tomoko Matsumoto, Yoshiki Kawabe, Takehisa Kitazawa, Midori Shima
BACKGROUND: Emicizumab is an anti-factor (F)IXa/X bispecific monoclonal antibody (mAb), mimicking the Factor (F)VIIIa cofactor activity. Emicizumab does not require activation by thrombin and its shortening effect on the activated partial prothrombin time (aPTT) is more pronounced than that of Factor (F)VIII. aPTT-based FVIII activity (FVIII:C) and FVIII inhibiter titer measurements are influenced by the presence of emicizumab. AIM: To establish a reliable aPTT-based assay to measure FVIII in the presence of emicizumab...
May 7, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29732985/treatment-of-chronic-articular-pain-in-adults-with-hemophilia
#7
E Carlos Rodriguez-Merchan
INTRODUCTION: Adult people with hemophilia (PWH) mainly use Factor VIII/Factor IX (FVIII/FIX) to lessen chronic articular pain, followed in frequency by nonsteroidal anti-inflammatory drugs. Analgesics are used by only one-third of adult PWH. Limitations in activities of daily living are encountered in the large majority of PWH, and most describe pain as affecting their state of mind. A review of the literature on their treatment is important because chronic pain in adult PWH is often undertreated or wrongly treated, causing psychological problems for these patients...
May 3, 2018: Cardiovascular & Hematological Disorders Drug Targets
https://www.readbyqxmd.com/read/29723894/anxiety-related-bleeding-and-thrombosis
#8
Silvia Hoirisch-Clapauch
Anxiety, a normal response to stressful situations, is characterized by increased levels of factor VIII, fibrinogen, and von Willebrand factor, and by enhanced platelet aggregability. One would expect acute anxiety to be a prothrombotic state, but since acute mental stress induces tissue plasminogen activator (tPA) release from endothelial and chromaffin cells, fibrinolysis counteracts procoagulant stimuli. It could be said that procoagulant changes accompanying the fight-or-flight response reduce the risk of bleeding in case of potential injuries, while activation of fibrinolysis counteracts activation of hemostasis to prevent intravascular thrombus formation before injuries occur...
May 3, 2018: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/29723892/treatment-regimens-with-bypassing-agents-in-patients-with-hemophilia-a-and-inhibitors-a-survey-from-the-italian-association-of-hemophilia-centers-aice
#9
Antonio Coppola, Massimo Franchini, Giancarlo Castaman, Elena Santagostino, Cristina Santoro, Rita Carlotta Santoro, Massimo Morfini, Giovanni Di Minno, Angiola Rocino
The development of neutralizing antibodies (inhibitors) against infused factor VIII currently represents the main complication of replacement therapy in patients with severe hemophilia A. Inhibitors, indeed, particularly high-titer inhibitors (>5 BU/mL), greatly complicate the management of bleeding, exposing patients to an increased morbidity and mortality risk, thus representing a significant burden for physicians of Hemophilia Treatment Centers (HTCs). Although bypassing agents (i.e., activated prothrombin complex concentrate [APCC] and recombinant activated factor VII [rFVIIa]) are available for the treatment and prevention of bleeding in inhibitor patients, their efficacy, safety, and cost-benefit outcomes are poorly known in the long term and should be further improved...
May 3, 2018: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/29696077/investigation-of-procoagulant-activity-in-extracellular-vesicles-isolated-by-differential-ultracentrifugation
#10
Thøger Nielsen, Anne Flou Kristensen, Shona Pedersen, Gunna Christiansen, Søren Risom Kristensen
Tissue factor (TF) is the main initiator of coagulation and procoagulant phospholipids (PPL) are key components in promoting coagulation activity in blood. Both TF and PPL may be presented on the surface of extracellular vesicles (EVs), thus contributing to their procoagulant activity. These EVs may constitute a substantial part of pathological hypercoagulability that is responsible for triggering a higher risk of thrombosis in certain patients. The aim of this study was to describe a model system for the isolation of EVs required for investigating their effect on coagulation...
2018: Journal of Extracellular Vesicles
https://www.readbyqxmd.com/read/29681200/cost-analysis-of-plasma-derived-factor-viii-von-willebrand-factor-vs-recombinant-factor-viii-for-treatment-of-previously-untreated-patients-with-severe-hemophilia-a-in-the-united-states
#11
Ellis J Neufeld, Robert F Sidonio, Ken O'Day, M Chris Runken, Kellie Meyer, Jeffrey Spears
BACKGROUND: Inhibitor development to factor VIII (FVIII) hemophilia therapy results in increased complications and substantial economic costs. The SIPPET study, the first randomized controlled trial to compare the immunogenicity of plasma-derived FVIII (pdFVIII)/von Willebrand factor (VWF) and recombinant-DNA-derived FVIII (rFVIII), demonstrated higher inhibitor rates in previously untreated patients (PUPs) treated with rFVIII than in PUPs treated with pdFVIII/VWF. OBJECTIVE: To quantify the economic impact of treating PUPs with pdFVIII/VWF versus rFVIII...
April 21, 2018: Journal of Medical Economics
https://www.readbyqxmd.com/read/29674503/dexamethasone-promotes-durable-factor-viii-specific-tolerance-in-hemophilia-a-mice-via-thymic-mechanisms
#12
Maria T Georgescu, Paul C Moorehead, Alice S van Velzen, Kate Nesbitt, Birgit M Reipert, Katharina N Steinitz, Maria Schuster, Christine Hough, David Lillicrap
The development of inhibitory antibodies to factor VIII is the most serious complication of replacement therapy in hemophilia A. Activation of the innate immune system during exposure to this protein contributes to inhibitor development. However, avoidance of factor VIII exposure during innate immune system activation by external stimuli (e.g. vaccines) has not been consistently shown to prevent inhibitors. We hypothesized that dexamethasone, a drug with potent anti-inflammatory effects, could prevent inhibitors by promoting immunologic tolerance to factor VIII in hemophilia A mice...
April 19, 2018: Haematologica
https://www.readbyqxmd.com/read/29665204/factor-viii-products-and-inhibitor-development-in-previously-treated-patients-with-severe-or-moderately-severe-haemophilia-a-a-systematic-review
#13
Shermarke Hassan, Antonino Cannavò, Samantha Gouw, Frits R Rosendaal, Johanna van der Bom
BACKGROUND: Patients with severe haemophilia A who have been treated extensively with factor VIII (FVIII) products face a low but potentially serious risk of inhibitor development. It is unknown why these patients break immunological tolerance and data on product-related immunogenicity is scarce. AIMS: To summarize the currently available evidence on the relationship between inhibitor development and recombinant FVIII product type in previously treated patients with severe haemophilia A...
April 17, 2018: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/29662861/global-hemostatic-assay-of-different-target-procoagulant-activities-of-factor-viii-and-factor-ix
#14
Ki-Young Yoo, Soo-Young Jung, Sung-Ho Hwang, Su-Min Lee, Jong-Ho Park, Hyun-Ja Nam
Background: Korean National Health Insurance reimburses factor VIII (FVIII) and factor IX (FIX) clotting factor concentrate (CFC) infusions to discrepant activity levels, allowing elevation of FVIII activity to 60 IU/dL and FIX to 40 IU/dL. We aimed to assess hemostatic response to these target levels using global hemostatic assays. Methods: We enrolled 34 normal healthy men, 34 patients with hemophilia A, and 36 with hemophilia B, with residual factor activity of 3 IU/dL or less and without inhibitors...
March 2018: Blood Research
https://www.readbyqxmd.com/read/29652676/immune-tolerance-induction-rescue-with-turoctocog-alfa-in-a-poor-risk-haemophilia-a-inhibitor-young-child-the-history-of-a-success
#15
Ezio Zanon, Marta Milan, Samantha Pasca
: The development of alloantibodies against the replacement of Factor VIII (FVIII) is the major complication in haemophilia A treatment. The gold standard to eradicate inhibitors is the immune tolerance induction (ITI), but in some cases it fails requiring another immune tolerance, defined ITI rescue (ITI-R), using a different concentrate, even though it is still debated. We report a successful case of a poor risk (titre of inhibitor at start of ITI > 10 BU/ml, peak titre on ITI > 200 BU/ml, >2 years since the inhibitor diagnosis) haemophilia A child treated with a high-dose regimen (200 UI/kg/day) turoctocog-alfa after a failed first-line ITI with octocog-alfa lasting 29 months...
April 12, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29614522/contribution-of-factor-viii-a2-domain-residues-400-409-to-a-factor-x-interactive-site-in-the-factor-xase-complex
#16
Masahiro Takeyama, Keiji Nogami, Kana Sasai, Shoko Furukawa, Midori Shima
The link between factor (F)VIII and FX is essential for optimum activity of the tenase complex. The interactive site(s) in FVIII for FX remains to be completely clarified, however. We investigated the FVIII A2 domain-FX association that was speculated from inhibitory mechanism(s) by an anti-A2 autoantibody. SDS-PAGE demonstrated that the purified inhibitor IgG recognizing residues 373-562 blocked FXa cleavage at Arg372 in FVIII, and surface-plasmon resonance (SPR)-based assays showed that intact A2 subunit directly bound to FX ( K d ; 63 nM)...
April 3, 2018: Thrombosis and Haemostasis
https://www.readbyqxmd.com/read/29611182/factor-f-viii-viia-enhances-global-haemostatic-function-in-the-co-presence-of-bypassing-agents-and-fviii-among-patients-with-haemophilia-a-with-inhibitor
#17
Keiji Nogami, Tomoko Matsumoto, Koji Yada, Kenichi Ogiwara, Shoko Furukawa, Yasuaki Shida, Masahiro Takeyama, Midori Shima
Bypassing therapy is essential for the haemostatic management of patients with haemophilia A with inhibitor (PWHA-inh), but the therapeutic effects are inconsistent. We previously reported that activated prothrombin complex concentrates (aPCC) activated factor (F)VIIIin vitro, and was mediated mainly by the activated FVII (FVIIa) contained in aPCC. We have extended those studies to assess global coagulation in whole blood from 18 PWHA-inh in the co-presence of aPCC and FVIII using Ca2+ -triggered rotational thromboelastometry...
April 2, 2018: British Journal of Haematology
https://www.readbyqxmd.com/read/29608457/can-the-plasmaderived-factor-viii-still-play-a-role-in-the-treatment-of-acquired-hemophilia-a-at-the-time-of-new-drugs
#18
Samantha Pasca, Vincenzo De Angelis, Marta Milan, Ezio Zanon
: Bypassing agents are the first-line therapy in the treatment of acquired hemophilia A (AHA), but not the only one. Other options as recombinant porcine factor VIII or plasmaderived concentrates (pdFVIII) are available to clinicians. Aim of this study was to evaluate whether the pdFVIII can still play a role in the treatment of AHA, and which patients could benefit from this therapy. All patients with AHA, presenting severe cardiovascular comorbidities, and treated with pdFVIII with or without von Willebrand factor (vWF), referred to two different hospitals, were initially considered...
March 31, 2018: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/29606934/vedolizumab-treatment-for-ulcerative-colitis-in-an-elderly-multimorbid-patient-with-hemophilia-a
#19
Holger Schäffler, Astrid Huth, Georg Lamprecht, Olaf Anders
The treatment of inflammatory bowel diseases (IBD) can be challenging, especially in elderly multimorbid patients. Since incidence and prevalence rates of IBD are rising steadily, treatment of older patients with relevant and also rare comorbidities will be of increasing relevancy for caregivers. Here we report on a 74-year-old multimorbid patient with severe ulcerative colitis (UC) and hemophilia A. Because of the chronic active disease, therapy with a tumor necrosis factor-α inhibitor was started. He suffered from a severe infectious complication (pneumonia) under therapy with infliximab...
September 2017: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/29606701/rbl2-p130-is-a-direct-akt-target-and-is-required-to-induce-apoptosis-upon-akt-inhibition-in-lung-cancer-and-mesothelioma-cell-lines
#20
Francesca Pentimalli, Iris M Forte, Luca Esposito, Paola Indovina, Carmelina A Iannuzzi, Luigi Alfano, Caterina Costa, Daniela Barone, Gaetano Rocco, Antonio Giordano
The retinoblastoma (RB) protein family includes RB1/p105, RBL1/p107, and RBL2/p130, which are key factors in cell-cycle regulation and stand at the crossroads of multiple pathways dictating cell fate decisions. The role of RB proteins in apoptosis is controversial because they can inhibit or promote apoptosis depending on the context, on the apoptotic stimuli and on their intrinsic status, impacting on the response to antitumoral treatments. Here we identified RBL2/p130 as a direct substrate of the AKT kinase, a key antiapoptotic factor hyperactive in multiple cancer types...
April 2, 2018: Oncogene
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