Global coagulation function assessed by rotational thromboelastometry predicts coagulation-steady state in individual hemophilia A patients receiving emicizumab prophylaxis.

Global coagulation function assessed by rotational thromboelastometry predicts coagulation-steady state in individual hemophilia A patients receiving emicizumab prophylaxis. Int J Hematol. 2019 Jun 28;: Authors: Yada K, Nogami K, Ogiwara K, Shida Y, Furukawa S, Yaoi H, Takeyama M, Kasai R, Shima M Abstract Emicizumab is a bispecific antibody to factor (F) IX/IXa and FX/FXa, which mimics FVIIIa cofactor function. Emicizumab prophylaxis significantly decreases bleeding events for patients with hemophilia A (PwHA). However, global hemostatic monitoring in emicizumab-treated PwHA remains poorly investigated. Using rotational thromboelastometry (ROTEM), we evaluated coagulation potentials of whole blood samples from seven emicizumab-treated PwHA who participated in ACE001JP/ACE002JP studies. Dose-dependent coagulation-enhancing effects of emicizumab to whole blood from PwHA mixed with an anti-FVIII C2 antibody in vitro were evident by non-activated ROTEM analysis (NATEM). The relationship between FVIII levels and NATEM parameters in PwHA not participating in the clinical trials demonstrated that CT + CFT inversely correlated with FVIII levels. These parameters were defined as NATEM-based grading of coagulation potential; 'T1' (FVIII 
Source: International Journal of Hematology - Category: Hematology Authors: Tags: Int J Hematol Source Type: research

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Authors: Kizilocak H, Young G Abstract Hemophilia A and B are inherited bleeding disorders characterized by deficiency or dysfunction of coagulation protein factors VIII and IX, respectively. Recurrent joint and muscle bleeds are the major clinical manifestations. Replacement therapy with clotting factors, either at the time of bleeding or as part of a prophylactic regimen, is adapted to individual patient needs. The major complication of therapy is the development of neutralizing antibodies. In response, researchers have developed novel agents to both reduce the treatment burden and prevent bleeding regardless of ...
Source: Clinical Advances in Hematology and Oncology - Category: Cancer & Oncology Tags: Clin Adv Hematol Oncol Source Type: research
Authors: Wan Ab Rahman WS, Abdullah WZ, Husin A, Nik Mohd Hassan NFF, Hassan MN, Zulkafli Z Abstract INTRODUCTION: Acquired haemophilia A (AHA) is a rare acquired bleeding disorder caused by polyclonal immunoglobulin G autoantibodies against clotting factor VIII (FVIII). The incidence was reported to be rare occurring in 0.2- 4 cases/million/year. Patients may present with different clinical manifestations to various specialties. Early recognition of the disease contributes to favourable clinical outcome. CASE SERIES: Here, we reported five cases of this disorder with different clinical presentations from two t...
Source: Malaysian Journal of Pathology - Category: Pathology Tags: Malays J Pathol Source Type: research
NHS England is to fund a life-changing treatment for thousands of people with severe haemophilia, which will dramatically cut their risk of life-threatening bleeds and reduce treatment time.
Source: NHS Networks - Category: UK Health Source Type: news
Rare clotting factor (F) deficiency is a deficiency of one or more of coagulation factors other than FVIII, FIX and vonWillebrand (FI, FII, FV, FV + FVIII, FVII, FIX, FX, FXI and FXIII) that cause bleeding disorders and are inherited as autosomal recessive. Descriptive study was conducted in Hemophilia Centre, Khartoum, Sudan. The medical files of pediatric patients presented to the center were reviewed retrospectively. Forty-seven patients (male : female ratio = 1.2 : 1) were included. The majority (93.6%) have parental history of consanguinity and around one third (31.9%) have family history of bleeding d...
Source: Blood Coagulation and Fibrinolysis - Category: Hematology Tags: ORIGINAL ARTICLES Source Type: research
We report development of an inhibitor in a child with severe hemophilia B, and subsequent immune tolerance induction using an extended desensitization protocol with the addition of immunosuppression. This case highlights successful management of a rare complication in a rare bleeding disorder and the need for additional investigation into this infrequent and clinically challenging occurrence.
Source: Blood Coagulation and Fibrinolysis - Category: Hematology Tags: CASE REPORTS Source Type: research
Conclusion: These patients with haemophilia are in general reasonably empowered on account of their personal experiences and their interaction with the healthcare providers. Current widespread access to information makes it easier to step up educational interventions in patients at lower haemorrhagic risk. PMID: 31393181 [PubMed - as supplied by publisher]
Source: Expert Review of Hematology - Category: Hematology Tags: Expert Rev Hematol Source Type: research
Hemophilia A and Hemophilia B are the most common of the severe bleeding disorders and are caused by a deficiency in blood clotting factor VIII or factor IX respectively. Factor replacement therapy has been the cornerstone of treatment to treat life threatening bleeds and prevent joint disease. The treatment of hemophilia has evolved tremendously over the past five decades from fresh frozen plasma as the only available therapy to more specific plasma-derived and recombinant-derived factor replacement.
Source: Transfusion and Apheresis Science - Category: Hematology Authors: Source Type: research
Factor IX:C (FIX:C) levels vary in hemophilia B carriers even in pedigrees with a unifying genetic defect. Analyzing the balance between pro-and anticoagulants might increase our understanding of carriers' bleeding potential.
Source: Thrombosis Research - Category: Hematology Authors: Tags: Full Length Article Source Type: research
Abstract Several lines of evidence seem to suggest that the clinical features of hemophilia A and B patients may differ in several points. Genetic background shows a prevalence of missense mutations in hemophilia B compared to the high percentage of mutations leading to no protein synthesis in hemophilia A. As a consequence, and also probably because factor IX is smaller than factor VIII, with less antigenic epitopes, the risk of inhibitor is significantly lower in hemophilia B. Although at presentation the two disorders show overlapping features as to age at first bleeding episode and treatment, data from interna...
Source: Haematologica - Category: Hematology Authors: Tags: Haematologica Source Type: research
Over the past decades hemophilia has been transformed from a debilitating disease to a manageable condition. However, the current treatment options are expensive, complex, and inaccessible to a large portion of the global population. Moreover, the development of antibodies to replacement factors, termed inhibitors, is a common complication that not only renders conventional prophylaxis regimens ineffective but also increase the annual bleeding rate in affected patients. Fortunately, much progress has been made toward developing a curative gene therapy treatment for hemophilia and these efforts have led to a series of human...
Source: Transfusion and Apheresis Science - Category: Hematology Authors: Source Type: research
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