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https://www.readbyqxmd.com/read/27913544/treatment-of-rare-factor-deficiencies-in-2016
#1
Flora Peyvandi, Marzia Menegatti
Rare bleeding disorders (RBDs) are a heterogeneous group of coagulation disorders characterized by fibrinogen, prothrombin, factors V, VII, X, XI, or XIII (FV, FVII, FX, FXI, or FXIII, respectively), and the combined factor V + VIII and vitamin K-dependent proteins deficiencies, representing roughly 5% of all bleeding disorders. They are usually transmitted as autosomal, recessive disorders, and the prevalence of the severe forms could range from 1 case in 500 000 for FVII up to 1 in 2-3 million for FXIII in the general population...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913543/hemophilia-and-inhibitors-current-treatment-options-and-potential-new-therapeutic-approaches
#2
Shannon L Meeks, Glaivy Batsuli
The immune response to infused factor concentrates remains a major source of morbidity and mortality in the treatment of patients with hemophilia A and B. This review focuses on current treatment options and novel therapies currently in clinical trials. After a brief review of immune tolerance regimens, the focus of the discussion is on preventing bleeding in patients with hemophilia and inhibitors. Recombinant factor VIIa and activated prothrombin complex concentrates are the mainstays in treating bleeds in patients with inhibitors...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27913541/what-is-the-role-of-an-extended-half-life-product-in-immune-tolerance-induction-in-a-patient-with-severe-hemophilia-a-and-high-titer-inhibitors
#3
Maissaa Janbain, Steven Pipe
A 10-year-old boy presents with a history of severe hemophilia A and high-titer inhibitor that had failed high-dose immune tolerance induction (ITI) with a recombinant factor VIII (rFVIII) product and a plasma-derived FVIII product. You are asked by his mother whether he should be tried on ITI with an extended half-life product, in particular, consideration of a rFVIIIFc concentrate.
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27902587/influence-of-factor-viii-level-and-its-inhibitor-titer-on-the-therapeutic-response-to-corticosteroids-alone-in-the-management-of-acquired-hemophilia-a-retrospective-single-center-study
#4
Mathieu Vautier, Hubert de Boysson, Christian Creveuil, Yohan Repesse, Annie Borel-Derlon, Xavier Troussard, Gandhi L Damaj, Boris Bienvenu, Philippe Gautier, Achille Aouba
The treatment of acquired hemophilia (AH) involves discussing whether corticosteroids should be administered alone or combined with immunosuppressant drugs, which increase the risk of infection especially in elderly patients and/or those with autoimmunity or neoplastic diseases, who represent the target population of the disease. Prognostic factors highlighting adequate responses to corticosteroids alone must be identified for satisfactory clinical response and lower infectious risk.We aimed to evaluating the efficacy of corticosteroids alone in the management of AH depending on factor VIII (FVIII, ≥ or <1 IU/dL) levels and/or inhibitor (INH, ≤ or >20 Bethesda units per milliliter [BU/mL]) titer...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27891721/extended-half-life-pegylated-full-length-recombinant-factor-viii-for-prophylaxis-in-children-with-severe-haemophilia-a
#5
E S Mullins, O Stasyshyn, M T Alvarez-Román, D Osman, R Liesner, W Engl, M Sharkhawy, B E Abbuehl
INTRODUCTION: Primary factor VIII (FVIII) prophylaxis is the optimal treatment in children with severe haemophilia A. They are expected to benefit from extended half-life (T1/2 ) FVIII coverage by reduced infusion frequency while maintaining haemostatic efficacy. AIMS: To determine immunogenicity, pharmacokinetics (PK), efficacy, safety and quality of life of prophylaxis with a polyethylene glycol (peg)-ylated FVIII (BAX 855) based on full-length recombinant FVIII (ADVATE) in paediatric previously treated patients (PTPs) with severe haemophilia A...
November 27, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27885373/a-new-era-of-treatment-for-patients-with-haemophilia-a
#6
Robert Klamroth
Treatment and prevention of bleeding episodes in patients with severe haemophilia A require frequent intravenous injection of factor VIII. Inhibitory antibodies against factor VIII occur in approximately 30 % of these patients during the first exposure days and immune tolerance induction to eradicate the inhibitor is challenging. Prevention of bleeds in patients with haemophilia A and inhibitors is less effective and there is ongoing research for alternative treatment options. A promising approach in 2016 is the development of emicizumab (ACE910), a bi-specific IgG antibody to factor IXa and factor X, that mimics the cofactor function of factor VIII...
November 25, 2016: Hämostaseologie
https://www.readbyqxmd.com/read/27885231/two-elderly-patients-with-difficult-to-treat-acquired-hemophilia-a
#7
Makoto Saito, Masanobu Morioka
We herein report the cases of two elderly patients with acquired hemophilia A (AHA) for whom treatment was difficult.An 89-year-old woman (Case 1) was admitted to our department with subcutaneous hemorrhage and melena. Her activated partial thromboplastin time (APTT), factor VIII activity, and factor VIII inhibitor level were 127.7 seconds, 1.0%, and 48 BU/mL, respectively, which was suggestive of AHA. The administration of prednisolone (PSL 0.5 mg/kg) was initiated. After 3 weeks, PSL was combined with cyclophosphamide (CPA 50 mg)...
2016: Nihon Ronen Igakkai Zasshi. Japanese Journal of Geriatrics
https://www.readbyqxmd.com/read/27878207/plasma-derived-versus-recombinant-factor-concentrates-in-pups-a-never-ending-debate
#8
Erik Berntorp
Inhibitor development in haemophilia is a serious complication to treatment with factor concentrates. Since the advent of more pure products, especially developed using recombinant DNA technology, some studies have shown an increased incidence of inhibitors in previously untreated patients (PUPs) receiving recombinant products whereas plasma-derived concentrates sometimes have been claimed to have a protective role, probably due to the content of von Willebrand factor (VWF). In fact, experiments indicate that the VWF may block uptake of factor VIII into macrophages for further processing to the immune system...
November 23, 2016: Hämostaseologie
https://www.readbyqxmd.com/read/27868337/enhanced-uptake-of-blood-coagulation-factor-viii-containing-immune-complexes-by-antigen-presenting-cells
#9
R B Hartholt, A Wroblewska, E Herczenik, I Peyron, A Ten Brinke, T Rispens, M A Nolte, E Slot, J W Claassens, F Nimmerjahn, J S Verbeek, J Voorberg
BACKGROUND: A major complication in the treatment of hemophilia A is the development of inhibitory antibodies targeting coagulation factor VIII (FVIII). Eradication of these inhibitors can be established by immune tolerance induction (ITI) which consists of daily administration of high dosages of FVIII. FVIII immune complexes (FVIII-IC) could be formed following FVIII infusion in patients with pre-existing anti-FVIII antibodies. OBJECTIVES: Here we studied endocytosis of FVIII-IC by bone marrow derived dendritic cells (BMDCs)...
November 21, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27862687/anti-factor-viii-antibodies-in-brothers-with-haemophilia-a-share-similar-characteristics
#10
J Kahle, A Orlowski, D Stichel, J F Healey, E T Parker, S M Donfield, J Astermark, E Berntorp, P Lollar, D Schwabe, C Königs
INTRODUCTION: The development of neutralizing antibodies (inhibitors) against coagulation factor VIII (FVIII) is currently the most serious complication for patients with haemophilia A undergoing FVIII replacement therapy. Several genetic factors have been acknowledged as risk factors for inhibitor development. AIM: To analyze the influence of genetic factors on the nature of the humoral immune response to FVIII in eight brother pairs with inhibitors. METHODS: The domain specificity of FVIII-specific IgG was analysed by antibody binding to FVIII fragments and homologue-scanning mutagenesis (HSM)...
November 8, 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27853081/unusual-initial-manifestation-of-acquired-hemophilia-a-a-normal-activated-partial-thromboplastin-time-intramuscular-hematoma-and-cerebral-hemorrhage
#11
Nobuaki Tsuyama, Toshihisa Ichiba, Hiroshi Naito
We herein present a case of acquired hemophilia A with a normal activated partial thromboplastin (aPTT), intramuscular hematoma and cerebral hemorrhage occurring in a 73-year-old man. The patient visited our emergency department with gait disturbance, pain and swelling in his right leg. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed intramuscular hematoma and intracranial hemorrhage. The results of initial coagulation studies were normal, but repeated coagulation studies revealed an isolated prolongation of the aPTT...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27852679/acquired-haemophilia-a-an-unusual-postoperative-complication
#12
Sumant Arora, Gaurav Goyal, Rehan Sarmad, Kenneth J Wool
An African-American man aged 65 years with multiple malignancies in remission was admitted for small bowel obstruction. He was treated with laparotomy following failure of conservative management. Postoperatively, he developed intra-abdominal bleed, which persisted, despite surgical haematoma evacuation. Further haematological workup revealed isolated prolongation of activated partial thromboplastin time (aPTT) with reduced factor VIII (FVIII) activity and raised FVIII inhibitor titre. Assuming acquired haemophilia A (AHA), FVIII inhibitor bypassing activity and corticosteroids were started with subsequent resolution of the bleeding from the surgical site...
November 16, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27843336/treatment-of-bleeding-in-acquired-hemophilia-a-with-the-proper-administration-of-recombinant-activated-factor-vii-single-center-study-of-7-cases
#13
Makoto Saito, Minoru Kanaya, Koh Izumiyama, Akio Mori, Tatsuro Irie, Masanori Tanaka, Masanobu Morioka, Masahiro Ieko
Recombinant activated factor VII (rFVIIa) is the bypassing agent used in the first-line hemostatic therapy for acquired hemophilia A (AHA); however, the occurrence of thrombotic complications in rFVIIa-treated AHA patients was recently reported to be 2.9-6.5%. Therefore, the investigation of the proper administration of rFVIIa for AHA is needed. In the present study, we retrospectively investigated the clinical features of AHA with regards to the use of rFVIIa (presence or absence of use and total amount) in 7 AHA patients encountered in our department for 7 years between January 2008 and December 2014...
2016: International Journal of General Medicine
https://www.readbyqxmd.com/read/27832514/analysis-of-the-japanese-subgroup-in-leopold-ii-a-phase-2-3-study-of-bay-81-8973-a-new-recombinant-factor-viii-product
#14
Teruhisa Fujii, Hideji Hanabusa, Midori Shima, Takeshi Morinaga, Katsuyuki Fukutake
BAY 81-8973, a new full length recombinant FVIII product, has been developed for prophylaxis and on-demand therapy in patients with hemophilia A. LEOPOLD II was a phase 2/3 study comparing prophylaxis versus on-demand treatment with BAY 81-8973. The analysis herein evaluated the clinical profile in Japanese subjects enrolled in LEOPOLD II. The LEOPOLD II was an open-label randomized crossover study. Our analysis evaluated the efficacy using the annualized bleeding rate, safety, and pharmacokinetics in Japanese subjects with severe hemophilia A enrolled in LEOPOLD II...
November 10, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27825181/blood-group-o-protects-against-inhibitor-development-in-severe-hemophilia-a-patients
#15
Massimo Franchini, Antonio Coppola, Carlo Mengoli, Gianna Franca Rivolta, Federica Riccardi, Giovanni Di Minno, Annarita Tagliaferri
Increasing evidence supports the link between ABO(H) blood group determinants and hemostasis. In particular, the ABO-related different glycosylation patterns of von Willebrand factor strongly influence its clearance and functional levels, and this may contribute to the inter-individual variations in the half-life of infused Factor VIII (FVIII) in hemophilia A (HA) patients. We investigated the role of ABO blood groups in regulating FVIII immunogenicity by evaluating their distribution in patients with severe (FVIII < 1 IU/dL) HA according to inhibitor development and other known relevant factors...
November 8, 2016: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/27816852/prevention-of-inhibitor-development-in-hemophilia-a-in-2016-a-glimpse-into-the-future
#16
Massimo Franchini, Giuseppe Lippi
Thanks to considerable progresses made over the last 30years, hemophilia benefits from the most efficacious and safe treatment among the many monogenic inherited disorders. The most challenging complication of replacement therapy in hemophilia A is the occurrence of alloantibodies against infused factor VIII (FVIII), thus predisposing the patients to increased morbidity and disability. Extensive research in this field has definitively unraveled that development of inhibitors in hemophilia A is a complex and multifactorial process, in which inherited and environmental factors dynamically interact...
October 27, 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27795652/-antihemophilic-factor-is-not-the-only-answer-for-all-factor-viii-deficiencies-case-report-of-odontogenic-infection-in-a-patient-with-hemophilia-a-complicated-by-factor-viii-inhibitors-and-managed-by-transfusion-of-antihemophilic-factor-and-factor-viii-inhibitor
#17
K M Sudheesh, K S N Siva Bharani, H Y Kiran, Suresh Hanagavadi
Dental extraction in hemophiliacs with acquired inhibitors is always a risky procedure, which often presents a lot of problems associated with bleeding. A known case of hemophilia A complicated with factor VIII inhibitors and having odontogenic infection was successfully managed by transfusion of factor VIII inhibitor bypass activity (FEIBA) and antihemophilic factor. Past medical history was significant for multiple factor VIII transfusions. Bethesda assay done to identify inhibitors revealed low titer factor VIII inhibitors...
September 2016: Indian Journal of Dentistry
https://www.readbyqxmd.com/read/27789479/design-and-characterization-of-an-apc-specific-serpin-for-the-treatment-of-hemophilia
#18
Stéphanie G I Polderdijk, Ty E Adams, Lacramioara Ivanciu, Rodney M Camire, Trevor P Baglin, James A Huntington
Hemophilia is a bleeding disorder caused by deficiency in factors VIII or IX, the two components of the intrinsic Xase complex. Treatment with replacement factor can lead to the development of inhibitory antibodies, requiring the use of bypassing agents such as factor VIIa and factor concentrates. An alternative approach to bypass the Xase complex is to inhibit endogenous anticoagulant activities. Activated protein C (APC) breaks down the complex that produces thrombin by proteolytically inactivating factor Va...
October 27, 2016: Blood
https://www.readbyqxmd.com/read/27780008/severe-hemophilia-a-in-a-male-old-english-sheep-dog-with-a-c%C3%A2-t-transition-that-created-a-premature-stop-codon-in-factor-viii
#19
Jay N Lozier, Mark T Kloos, Elizabeth P Merricks, Nathaly Lemoine, Margaret H Whitford, Robin A Raymer, Dwight A Bellinger, Timothy C Nichols
Animals with hemophilia are models for gene therapy, factor replacement, and inhibitor development in humans. We have actively sought dogs with severe hemophilia A that have novel factor VIII mutations unlike the previously described factor VIII intron 22 inversion. A male Old English Sheepdog with recurrent soft-tissue hemorrhage and hemarthrosis was diagnosed with severe hemophilia A (factor VIII activity less than 1% of normal). We purified genomic DNA from this dog and ruled out the common intron 22 inversion; we then sequenced all 26 exons...
2016: Comparative Medicine
https://www.readbyqxmd.com/read/27774572/amiodarone-induced-retinal-neuronal-cell-apoptosis-attenuated-by-igf-1-via-counter-regulation-of-the-pi3k-akt-foxo3a-pathway
#20
Rifang Liao, Fengxia Yan, Zhuanping Zeng, Mohd Farhan, Peter Little, Remi Quirion, Lalit K Srivastava, Wenhua Zheng
Amiodarone (AM) is the most effective antiarrhythmic agent currently available. However, clinical application of AM is limited by its serious toxic adverse effects including optic neuropathy. The purpose of this study was to explore the effects of AM and to assess if insulin-like growth factor-1 (IGF-1) could protect retinal neuronal cells from AM-induced apoptosis, and to determine the molecular mechanisms underlying the effects. Accordingly, the phosphorylation/activation of Akt and FoxO3a were analyzed by Western blot while the possible pathways involved in the protection of IGF-1 were investigated by application of various pathway inhibitors...
October 24, 2016: Molecular Neurobiology
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