Akram Mesleh Shayeb, Chen Li, Guolian Kang, Ulrike M Reiss, Lama Elbahlawan
: Bleeding among critically ill paediatric haematology/oncology (CIPHO) patients leads to significant morbidity and mortality. Recombinant activated factor VII (rFVIIa) has shown some benefits in previous reported off-label use when conventional therapies have failed. However, data in CIPHO are lacking. We retrospectively studied (2006-2014) the efficacy and outcomes in CIPHO patients younger than 21 years who received at least one rFVIIa dose for bleeding in the ICU. Of 39 patients, the majority had leukaemia (59%), bone marrow transplantation (77%) and a life-threatening bleed (80%) with most common site being pulmonary haemorrhage (44%)...
August 21, 2020: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
Ryo Yanagiya, Kazunori Kanouchi, Tomomi Toubai, Akane Yamada, Keiko Aizawa, Yosuke Shiono, Satoshi Ito, Kenichi Ishizawa
Acquired factor V deficiency (AFVD) is a rare autoimmune bleeding disorder. Unlike acquired hemophilia, bypass therapies with recombinant activated factor VII and activated prothrombin complex concentrates are ineffective for severe bleeding due to AFVD. Although several treatment strategies have been attempted, a standard of care for severe hemorrhage induced by AFVD is lacking. Herein, we report a case of AFVD with severe bleeding that responded to plasma exchange (PE) combined with immunosuppression. We also reviewed previously reported AFVD cases with severe hemorrhage and suggest that PE may be an effective initial treatment for AFVD-induced severe hemorrhage...
August 12, 2020: Acta Haematologica
Sema Aylan Gelen, Nazan Sarper, Emine Zengin
BACKGROUND AND OBJECTIVES: In this retrospective report the aim was to present the experience about bleeding characteristics and management of minor surgeries in rare bleeding disorders (RBDs). METHODS: Twenty-six patients were included; with Factor (F) V, FV+VIII, VII, FXI deficiency and afibrinogenemia. Six of the patients were asymptomatic. RESULTS: Fifty-three percent of the patients suffered from mucosal bleeding. Life-threatening bleedings were observed only in the patients with afibrinogenemia and good hemostatic control could only be provided with plasma-derived (pd)-fibrinogen concentrate...
2020: Turkish Journal of Pediatrics
Stephen J Huddleston, Scott Jackson, Kathleen Kane, Nicholas Lemke, Andrew W Shaffer, Matthew Soule, Marshall Hertz, Sara Shumway, Stephen Qi, Tjorvi Perry, Rosemary Kelly
OBJECTIVE: The purpose of this study was to determine the relationship between blood product transfusion, with or without recombinant human activated factor VIIa, and survival after lung transplantation. DESIGN: Retrospective analysis of a single center with follow-up out to 6 years post-transplantation. SETTING: Single-center academic lung transplantation program. PARTICIPANTS: The study comprised 265 adult patients who underwent single or bilateral sequential lung transplantation from March 2011 to June 2017...
June 3, 2020: Journal of Cardiothoracic and Vascular Anesthesia
Georg Gelbenegger, Christian Schoergenhofer, Paul Knoebl, Bernd Jilma
Hemophilia A, characterized by absent or ineffective coagulation factor VIII (FVIII), is a serious bleeding disorder that entails severe and potentially life-threatening bleeding events. Current standard therapy still involves replacement of FVIII, but is often complicated by the occurrence of neutralizing alloantibodies (inhibitors). Management of patients with inhibitors is challenging and necessitates immune tolerance induction for inhibitor eradication and the use of bypassing agents (activated prothrombin complex concentrates or recombinant activated factor VII), which are expensive and not always effective...
July 27, 2020: Thrombosis and Haemostasis
Eriko Uchida, Kazutoshi Komori, Takashi Kurata, Masashi Taki, Kazuo Sakashita
Treating patients with hemophilia and inhibitors is often problematic. The presence of inhibitors negatively impacts the effectiveness of treatment to achieve hemostasis especially in patients with hemophilia B, owing mainly to allergic reactions to factor IX (FIX) concentrates and the low success rate of immune tolerance therapy. A 9-month-old boy had intracranial hemorrhage and was diagnosed with hemophilia B. After replacement therapy, he developed inhibitors and an allergic reaction to FIX. Prophylactic therapy was initiated with recombinant activated factor VII (rFVIIa) and later switched to pdFVIIa/factor X (FX; 120 μg/kg as the FVII dose, every other day) because of a recurrence of intracranial hemorrhage...
July 22, 2020: Acta Haematologica
Başak Koç Şenol, Bülent Zülfikar
Aim: Factor VII deficiency is one of the hereditary coagulation disorders that has autosomal reccessive inheritance and is observed relatively frequently (1/500 000). It is clinically heterogeneous, and may be asymptomatic or lead to life-threatening bleeding. Thus, there is no correlation between FVII activity and clinical findings. Plasma-derived and recombinant FVII concentrates are currently used for treatment. In countries where access to these products is lacking, fresh frozen plasma and prothrombin complex concentrates are also used, though they contain low amounts of factor FVII...
2020: Türk Pediatri Arşivi
Eun-Ju Lee, Laura Burey, Sharon Abramovitz, Maria T Desancho
INTRODUCTION: Inherited factor VII deficiency is the most common autosomal recessive inherited bleeding disorder, with an estimated incidence of one per 500 000 cases in the general population. Bleeding manifestations correlate poorly with circulating FVII levels. During pregnancy, increases in FVII levels can occur in women with mild-moderate FVII deficiencies but not in those with severe deficiency. AIM: We present five pregnant patients with FVII deficiency and describe the management during their pregnancies and peripartum periods...
June 26, 2020: Haemophilia: the Official Journal of the World Federation of Hemophilia
Hideo Wada, Katsuya Shiraki, Takeshi Matsumoto, Kohshi Ohishi, Hideto Shimpo, Motomu Shimaoka
INTRODUCTION: Physiological coagulation is considered to activate coagulation factor IX (FIX) by a small amount of tissue factor (TF) and activated coagulation factor VII (FVIIa) with the presence of platelets. A Clot waveform analysis (CWA) may be useful for evaluating physiological coagulation. MATERIAL AND METHODS: A CWA using a small amount of TF (CWA/sTF) was performed in platelet-rich plasma (PRP), platelet-poor plasma (PPP), several phospholipids (PLs) and patients with lupus anticoagulant (LA), idiopathic thrombocytopenic purpura (ITP) or inhibitor for FVIII...
June 10, 2020: Thrombosis Research
Yu Shimizu, Katsuhiro Tsuchiya, Norihiro Fujisawa
Background: Diffuse alveolar hemorrhage (DAH) is a rare and frequently life-threatening complication of a variety of conditions. DAH may result from coagulation disorders, inhaled toxins, or infections. We report a series of patients who developed DAH after receiving a tissue-type plasminogen activator (tPA) for acute cerebral infarction. We aimed to find risk factors of DAH in patients receiving tPA and the effectiveness of activated recombinant factor VII (rFVIIa) treatment for the same...
2020: Surgical Neurology International
Silvia Linari, Giancarlo Castaman
Emicizumab, a humanized, bi-specific, monoclonal antibody subcutaneously administered, mimicking the function of FVIIIa, represents a milestone in treatment of patients affected by hemophilia A complicated with inhibitors. The HAVEN 1 and 2 studies have clearly established its superiority compared to bypassing agents for routine prophylaxis in preventing or reducing bleeding episodes in adult and pediatric patients with inhibitors. However, its protection against bleeding is only partial, and concomitant use of a bypassing agent may be required with potential prothrombotic risk...
2020: Therapeutics and Clinical Risk Management
Muhammad Bashir Bello, Siti Nor Azizah Mahamud, Khatijah Yusoff, Aini Ideris, Mohd Hair-Bejo, Ben P H Peeters, Abdul Rahman Omar
Genotype VII Newcastle disease viruses are associated with huge economic losses in the global poultry industry. Despite the intensive applications of vaccines, disease outbreaks caused by those viruses continue to occur frequently even among the vaccinated poultry farms. An important factor in the suboptimal protective efficacy of the current vaccines is the genetic mismatch between the prevalent strains and the vaccine strains. Therefore, in the present study, an effective and stable genotype-matched live attenuated Newcastle disease virus (NDV) vaccine was developed using reverse genetics, based on a recently isolated virulent naturally recombinant NDV IBS025/13 Malaysian strain...
June 2, 2020: Vaccines
François Mullier, Marie-Sophie Paridaens, Jonathan Evrard, Justine Baudar, Maité Guldenpfennig, Celia Devroye, Laurence Miller, Bernard Chatelain, Sarah Lessire, Hugues Jacqmin
INTRODUCTION: We aimed at evaluating the performance of a new prothrombin time (PT) reagent (STA-NeoPTimal) with two other PT reagents (STA-Neoplastine R and STA-Neoplastine CI Plus) and the reference PT reagent used in our laboratory (ReadiPlasTin). METHODS: Evaluation consisted in intra- and interassay precision assessment, determination of sensitivity to unfractionated heparin (UFH) or enoxaparin in spiked samples and to direct oral anticoagulants (DOACs) in patients (n = 43)...
May 19, 2020: International Journal of Laboratory Hematology
Antonio Girolami, Mariano Paoletti, Silvia Ferrari, Diana Garcia
OBJECTIVE: To investigate a family with factor VII (FVII) deficiency from Argentina. PATIENTS AND METHODS: The proposita is a 14-year-old girl who presented with a mild to moderate bleeding tendency. Menorrhagia is controlled with periodical administration of small doses of recombinant FVII concentrate. The mother of the proposita has a similar bleeding tendency. RESULTS: FVII activity in both patients was 20% of normal; FVII antigen was 35% of normal...
May 12, 2020: Acta Haematologica
Andreas Tiede, Peter Collins, Paul Knoebl, Jerome Teitel, Craig Kessler, Midori Shima, Giovanni Di Minno, Roseline d'Oiron, Peter Salaj, Victor Jiménez-Yuste, Angela Huth-Kühne, Paul Giangrande
Acquired hemophilia A (AHA), a rare bleeding disorder caused by neutralizing autoantibodies against coagulation factor VIII (FVIII), occurs in both men and women without a previous history of bleeding. Patients typically present with an isolated prolonged activated partial thromboplastin time due to FVIII deficiency. Neutralizing antibodies (inhibitors) are detected using the Nijmegen-modified Bethesda assay. Approximately 10% of patients do not present with bleeding and, therefore, a prolonged activated partial thromboplastin time should never be ignored prior to invasive procedures...
July 2020: Haematologica
Maria-Isabel Bravo, Aida Raventós, Alba Pérez, Montserrat Costa, Todd Willis
BACKGROUND: Emicizumab is an alternative non-factor approach for treating patients with hemophilia A. However, there is a potential risk of thrombotic events when emicizumab is concomitantly administered with pro-hemostatic therapies. OBJECTIVES: To assess the hemostatic effect in vitro when a plasma-derived factor VIII concentrate containing von Willebrand factor (pdFVIII/VWF) was added to hemophilia A plasma (HAp) in combination with emicizumab. METHODS: HAp and HAp with FVIII inhibitors (HAp-i) samples with different concentrations of emicizumab (50 and 100 μg/mL) were combined with activated prothrombin complex concentrate (aPCC) at 0...
May 7, 2020: Journal of Thrombosis and Haemostasis: JTH
Siti Kurniawati, Ni Made Mertaniasih, Manabu Ato, Toshiki Tamura, Soedarsono Soedarsono, Aulanni'am Aulanni'am, Shigetarou Mori, Yumi Maeda, Tetsu Mukai
Mycobacterium tuberculosis ( M. tuberculosis ) is the causative agent of tuberculosis in human. One of the major M. tuberculosis virulence factors is early secretory antigenic target of 6-kDa (ESAT-6), and EccB5 protein encoded by eccB5 is one of its components. EccB5 protein is a transmembrane protein in ESX-5 system. The aim of this study is to explore the characteristics of wild-type EccB5 and its mutant form N426I. We expressed the EccB5 protein by cloning the mutant and wild-type eccB5 gene in Escherichia coli ( E...
2020: BioResearch Open Access
Juliana Perez Botero, Kristy Lee, Brian R Branchford, Paul F Bray, Kathleen Freson, Michele P Lambert, Minjie Luo, Shruthi Mohan, Justyne E Ross, Wolfgang Bergmeier, Jorge Di Paola
Glanzmann thrombasthenia (GT) is an autosomal recessive disorder of platelet aggregation caused by quantitative or qualitative defects in integrins αIIb and β3. These integrins are encoded by the ITGA2B and ITGB3 genes and form platelet glycoprotein (GP)IIb/IIIa, which acts as the principal platelet receptor for fibrinogen. Although there is variability in the clinical phenotype, most patients present with severe mucocutaneous bleeding at an early age. A classic pattern of abnormal platelet aggregation, platelet glycoprotein expression and molecular studies confirm the diagnosis...
April 2020: Haematologica
John Fitzgerald, Robert McMonnies, Aidan Sharkey, Peter L Gross, Keyvan Karkouti
This narrative review discusses the role of thrombin generation in coagulation and bleeding in cardiac surgery, the laboratory methods for clinical detection of impaired thrombin generation, and the available hemostatic interventions that can be used to improve thrombin generation. Coagulopathy after cardiopulmonary bypass (CPB) is associated with excessive blood loss and adverse patient outcomes. Thrombin plays a crucial role in primary hemostasis, and impaired thrombin generation can be an important cause of post-CPB coagulopathy...
June 2020: Canadian Journal of Anaesthesia, Journal Canadien D'anesthésie
Tami Livnat, Alfica Sehgal, Kun Qian, Huy Van Nguyen, Kate Madigan, Benny Sorensen, Gili Kenet
Antithrombin (AT) reduction has been shown to improve thrombin generation (TG) in haemophilia with or without inhibitors. As treatment with bypassing agents (BPAs) may be required in patients with breakthrough bleeding while receiving AT-lowering therapy, we assessed TG in platelet-poor plasma samples from haemophilia patients in the presence of BPA (recombinant activated factor VII [rFVIIa; 1.25 or 2.5 μg mL-1 ] or activated prothrombin complex concentrate [aPCC; 0.5 or 1 U mL-1 ]) and AT reduction (anti-AT antibody)...
May 2020: Blood Cells, Molecules & Diseases
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