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https://www.readbyqxmd.com/read/27920236/the-efficacy-of-recombinant-fviii-low-dose-prophylaxis-in-chinese-pediatric-patients-with-severe-hemophilia-a-a-retrospective-analysis-from-the-recare-study
#1
Wanru Yao, Juan Xiao, Xiaoling Cheng, Guoshuang Feng, Changgang Li, Xinsheng Zhang, Qun Hu, Weiqun Xu, Jing Sun, Renchi Yang, Xiaojing Li, Rongfu Zhou, Shimei Lian, Jian Gu, Junde Wu, Yongqiang Zhao, Runhui Wu
OBJECTIVE: This study explores the efficacy of recombinant factor VIII (rFVIII) low-dose prophylaxis in Chinese pediatric patients with severe hemophilia A from the Retrospective Study in Chinese Pediatric Hemophilia A Patients With rFVIII Contained Regular Prophylaxis (ReCARE) population. METHODS: This is additional analysis of the multicenter, retrospective ReCARE study, in which the annual bleeding rate (ABR), annual joint bleeding rate (AJBR), and safety of >12-week, low dose (10-30 IU/kg/wk) rFVIII prophylaxis divided into primary, secondary, and tertiary groups based on the joint status and joint bleeding history were analyzed...
December 5, 2016: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/27919309/cost-utility-analysis-of-primary-prophylaxis-compared-with-on-demand-treatment-for-patients-with-severe-hemophilia-type-a-in-colombia
#2
Héctor Eduardo Castro Jaramillo, Mabel Moreno Viscaya, Aurelio E Mejia
OBJECTIVES: This article presents a cost-utility analysis from the Colombian health system perspective comparing primary prophylaxis to on-demand treatment using exogenous clotting factor VIII (FVIII) for patients with severe hemophilia type A. METHODS: We developed a Markov model to estimate expected costs and outcomes (measured as quality-adjusted life-years, QALYs) for each strategy. Transition probabilities were estimated using published studies; utility weights were obtained from a sample of Colombian patients with hemophilia and costs were gathered using local data...
December 6, 2016: International Journal of Technology Assessment in Health Care
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/27904904/clinical-evaluation-of-glycopegylated-recombinant-fviii-efficacy-and-safety-in-severe-haemophilia-a
#4
Paul Giangrande, Tatiana Andreeva, Pratima Chowdary, Silke Ehrenforth, Hideji Hanabusa, Frank W G Leebeek, Steven R Lentz, Laszlo Nemes, Lone Hvitfeldt Poulsen, Elena Santagostino, Chur Woo You, Wan Hui Ong Clausen, Peter G Jönsson, Johannes Oldenburg
Turoctocog alfa pegol (N8-GP) is a novel glycoPEGylated extended half-life recombinant factor VIII (FVIII) product developed for prophylaxis and treatment of bleeds in patients with haemophilia A, to enable higher activity levels with less frequent injections compared with standard FVIII products. This phase III (NCT01480180), multinational, open-label, non-randomised trial evaluated the safety and clinical efficacy of N8-GP when administered for treatment of bleeds and for prophylaxis, in previously treated patients aged ≥12 years with severe haemophilia A...
December 1, 2016: Thrombosis and Haemostasis
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
#5
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/27898515/cost-effectiveness-analysis-of-pharmacokinetic-driven-prophylaxis-vs-standard-prophylaxis-in-patients-with-severe-haemophilia-a
#6
Sergio Iannazzo, Paolo A Cortesi, Roberto Crea, Katharina Steinitz, Lorenzo G Mantovani, Alessandro Gringeri
The objective of this study was to assess the cost-effectiveness of pharmacokinetic-driven prophylaxis in severe haemophilia A patients. A microsimulation model was developed to evaluate the cost-effectiveness of pharmacokinetic-driven prophylaxis vs. standard prophylaxis and estimate cost, annual joint bleed rate (AJBR), and incremental cost-effectiveness ratio over a 1-year time horizon for a hypothetical population of 10 000 severe haemophilia A patients. A dose of 30 IU/kg per 48 h was assumed for standard prophylaxis...
November 24, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27891721/extended-half-life-pegylated-full-length-recombinant-factor-viii-for-prophylaxis-in-children-with-severe-haemophilia-a
#7
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/27891624/quarantine-versus-pathogen-reduced-plasma-coagulation-factor-content-and-rotational-thromboelastometry-coagulation
#8
Oliver M Theusinger, David Goslings, Jan-Dirk Studt, Brigitte Brand-Staufer, Burkhardt Seifert, Donat R Spahn, Beat M Frey
BACKGROUND: Different types of fresh-frozen plasma (FFP) exist, and the concentrations of plasma proteins vary between individuals and blood groups. Furthermore, processing may also influence the content. Quarantine-stored plasma (qFFP) and plasma that was pathogen-reduced using blood-safety (Intercept) technology (piFFP) were analyzed regarding procoagulant and anticoagulant hemostasis proteins, including endogenous thrombin (thrombin-generation) potential (ETP). MATERIALS AND METHODS: Thirty-five samples of each type of FFP were analyzed using only male Blood Group O donors...
November 27, 2016: Transfusion
https://www.readbyqxmd.com/read/27882137/von-willebrand-factor-and-coagulation-factor-viii-in-moyamoya-disease-associated-with-graves-disease-a-case-report
#9
Shou-Chen Ren, Bao-Qin Gao, Wei-Li Yang, Wei-Xin Feng, Jian Xu, Shao-Wu Li, Yong-Jun Wang
The present study reported the case of a Chinese boy who was diagnosed with Moyamoya disease (MMD) associated with Graves' disease (GD). An overactivation of von Willebrand factor (vWF) and coagulation factor VIII (FVIII) was identified in the plasma of the patient. Thiamazole and metoprolol treatment was thus administrated. After 2 months of treatment, the patient's thyroid function returned to normal and the neurological symptoms improved gradually. At the same time, the activities of vWF and FVIII were depressed...
November 2016: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/27868395/genetic-diagnosis-in-hemophilia-a-from-southern-china-five-novel-mutations-and-one-preimplantation-genetic-analysis
#10
J Chen, J Wang, X Y Lin, Y W Xu, Z H He, H Y Li, S Q Chen, W Y Jiang
INTRODUCTION: As there is currently no complete cure for hemophilia A (HA), the identification of pathogenic mutations in factor VIII (FVIII) gene from HA patients and carriers, which can contribute to genetic counseling prenatal diagnosis, and preimplantation genetic diagnosis (PGD), is an important step to prevent HA. METHODS: A total of 14 unrelated Chinese HA subjects (FVIII activity <40%), 20 carrier subjects, three fetuses, and one PGD were included in this study...
November 21, 2016: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/27868369/evaluation-of-the-utility-of-the-isth-bat-in-haemophilia-carriers-a-multinational-study
#11
P D James, J Mahlangu, C Bidlingmaier, M E Mingot-Castellano, M Chitlur, P F Fogarty, A Cuker, M E Mancuso, P A Holme, J Grabell, N Satkunam, W M Hopman, P Mathew
INTRODUCTION: There has been increasing recognition in recent years that female carriers of haemophilia manifest abnormal bleeding; however, data on the use of bleeding assessment tools in this population are lacking. AIM: Our objective was to validate the ISTH-BAT in haemophilia carriers to describe bleeding symptoms and allow for comparisons with factor levels and other patient groups. METHODS: This was a prospective, observational, cross-sectional study performed by members of Global Emerging HEmostasis Panel (GEHEP)...
November 2016: Haemophilia: the Official Journal of the World Federation of Hemophilia
https://www.readbyqxmd.com/read/27868337/enhanced-uptake-of-blood-coagulation-factor-viii-containing-immune-complexes-by-antigen-presenting-cells
#12
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/27864149/mechanistic-insight-into-the-procoagulant-activity-of-tumor-derived-apoptotic-vesicles
#13
Morad-Rémy Muhsin-Sharafaldine, Bailey R Kennedy, Sarah C Saunderson, Catrin R Buchanan, Amy C Dunn, James M Faed, Alexander D McLellan
BACKGROUND: Chemotherapy induces the release of apoptotic vesicles (ApoV) from the tumor plasma membrane. Tumor ApoV may enhance the risk of thrombotic events in cancer patients undergoing chemotherapy. However, the relative contribution of ApoV to coagulation and the pathways involved remain poorly characterized. In addition, this study sets out to compare the procoagulant activity of chemotherapy-induced ApoV with their cell of origin and to determine the mechanisms of ApoV-induced coagulation...
November 15, 2016: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27862687/anti-factor-viii-antibodies-in-brothers-with-haemophilia-a-share-similar-characteristics
#14
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/27852679/acquired-haemophilia-a-an-unusual-postoperative-complication
#15
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/27848918/development-and-validation-of-technical-assays-of-von-willebrand-disease-in-routine-laboratory
#16
Sara Derdabi, Laurence Rozen, Carole Nagant, Anne Demulder
Von Willebrand disease (MW) is the most common constitutional bleeding disorders. It is caused by a quantitative or qualitative abnormality of the von Willebrand factor (VWF). The laboratory assessment of the disease combines a FVIII assay, and a determination of the antigen and activity of VWF. The analytical validation of VWF:Ag, VWF:Act, vWF:CB is reported in this work and demonstrates good test performance of all three assays, with a coefficient of variation lower than 10% for both the repeatability and reproducibility, stability with a deviation of less than 5% from the target value after six hours at room temperature...
December 1, 2016: Annales de Biologie Clinique
https://www.readbyqxmd.com/read/27833753/adherence-to-treatment-regimen-and-bleeding-rates-in-a-prospective-cohort-of-youth-and-young-adults-on-low-dose-daily-prophylaxis-for-severe-hemophilia-a
#17
Terry Mizrahi, Jean St-Louis, Nancy L Young, Francine Ménard, Nichan Zourikian, Evemie Dubé, Georges E Rivard
BACKGROUND: When availability and/or affordability of anti-hemophilic factor concentrates are limited, optimal prophylaxis regimens in severe hemophilia A (HA) remain to be determined. In selected situations, low-dose daily prophylaxis (LDDP) may be an effective and economical option. The goal of our study was to evaluate if subjects on a LDDP regimen could achieve adherence and good clinical outcome. METHODS: Seventeen subjects (age between 15.2 and 28.4) on LDDP suffering from severe/moderate HA were followed prospectively for 2 to 3 years as part of a health-related quality of life (HRQoL) study...
2016: BMC Hematology
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
#18
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/27827474/the-prothrombotic-state-associated-with-obesity-induced-hypertension-is-reduced-by-cocoa-and-its-main-flavanols
#19
G M Rabadán-Chávez, E Reyes-Maldonado, L Quevedo-Corona, N Paniagua-Castro, G Escalona-Cardoso, M E Jaramillo-Flores
BACKGROUND: Little is known about the effects of cocoa and its main flavanols on the prothrombotic state associated with the development of hypertension in diet-induced obesity models. PURPOSE: To evaluate the effects of cocoa powder, cocoa extract and their main flavanols on plasma biomarkers related to impaired coagulation and fibrinolysis and its association with hypertension and obesity-related metabolic disorders in rats fed a hypercaloric diet. METHODS: Male Wistar rats were randomly assigned to 7 treatment groups (n = 7): normal diet (ND); hypercaloric diet control group (HCD); HCD + cocoa powder (CO); HCD + cocoa extract (CO-EX); HCD + (-)-epicatechin (EPI); HCD + (+)-catechin (CAT); and HCD + procyanidin B2 (PB2)...
November 9, 2016: Food & Function
https://www.readbyqxmd.com/read/27825181/blood-group-o-protects-against-inhibitor-development-in-severe-hemophilia-a-patients
#20
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
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