Articles

Hemophilia A gene therapy via intraosseous delivery of factor VIII-lentiviral vectors

Current treatment of hemophilia A (HemA) patients with repeated infusions of factor VIII (FVIII; abbreviated as F8 in constructs) is costly, inconvenient, and incompletely effective. In addition, approximately 25...

Authors: Carol H. Miao

Thrombopoietin and its receptor in normal and neoplastic hematopoiesis

Thrombopoietin was posited to exist in 1958 and cloned in 1994, and in the ensuing two decades we have learned a great deal about the physiology and pathology of the primary regulator of thrombopoiesis. This p...

Authors: Kenneth Kaushansky

Characteristics of Taiwanese patients of PNH in the international PNH registry

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare and acquired hematopoietic stem cell disease, with florid clinical presentations. Although this disease has been characterized in the western countries, its ...

Authors: Wen-Chien Chou, Wei-Han Huang, Ming-Chung Wang, Chao-Sung Chang, Shih-Peng Yeh, Tzeon-Jye Chiou, Yeu-Chin Chen, Tseng-Hsi Lin and Ming-Ching Shen

Aspirin plus tirofiban inhibit the thrombosis induced by Russell’s viper venom

Thrombosis and coagulopathy are the commonest hematological manifestations of envenomation of Russell’s viper venom (RVV). Factor X is activated by a factor X-activating enzyme from Russell’s viper venom (RVV-...

Authors: Ren-Chieh Wu, Ping-Tse Chou and Li-Kuang Chen

De novo mutation and somatic mosaicism of gene mutation in type 2A, 2B and 2M VWD

Von Willebrand disease (VWD) is not uncommon in Taiwan. In type 2 or type 3 VWD hemorrhagic symptoms are severer and laboratory data relatively more distinctive. De novo mutation and somatic mosaicism of type 2 V...

Authors: Ming-Ching Shen, Ming Chen, Gwo-Chin Ma, Shun-Ping Chang, Ching-Yeh Lin, Bo-Do Lin and Han-Ni Hsieh

Comorbidities of cardiovascular disease and cancer in hemophilia patients

As life expectancy greatly increases in persons with hemophilia (PWH), more age-related diseases such as cancer and cardiovascular disease (CVD) emerge among this patient group. The aim of this study was to re...

Authors: Jiaan-Der Wang

Preimplantation genetic diagnosis of hemophilia A

Preimplantation genetic diagnosis (PGD) is a powerful tool to tackle the transmission of monogenic inherited disorders in families carrying the diseases from generation to generation. It currently remains a ch...

Authors: Ming Chen, Shun-Ping Chang, Gwo-Chin Ma, Wen-Hsian Lin, Hsin-Fu Chen, Shee-Uan Chen, Horng-Der Tsai, Feng-Po Tsai and Ming-Ching Shen

Different impact of factor VIII products on inhibitor development?

Inhibitors are the most serious side effect of haemophilia treatment; they occur in 25–30 % of all patients with severe haemophilia A. Over the last 2 decades, conflicting data on the impact of clotting produc...

Authors: H. Marijke van den Berg

Aspects of prophylactic treatment of hemophilia

Retrospective and prospective studies unambiguously show that prophylactic treatment of severe hemophilia A or B should be started as primary prophylaxis at 1–2 years’ of age and ideally before the first joint...

Authors: Rolf Ljung

Platelets and platelet adhesion molecules: novel mechanisms of thrombosis and anti-thrombotic therapies

Platelets are central mediators of thrombosis and hemostasis. At the site of vascular injury, platelet accumulation (i.e. adhesion and aggregation) constitutes the first wave of hemostasis. Blood coagulation, ...

Authors: Xiaohong Ruby Xu, Naadiya Carrim, Miguel Antonio Dias Neves, Thomas McKeown, Tyler W. Stratton, Rodrigo Matos Pinto Coelho, Xi Lei, Pingguo Chen, Jianhua Xu, Xiangrong Dai, Benjamin Xiaoyi Li and Heyu Ni

New horizon in platelet function: with special reference to a recently-found molecule, CLEC-2

Platelets play a key role in the pathophysiological processes of hemostasis and thrombus formation. However, platelet functions beyond thrombosis and hemostasis have been increasingly identified in recent year...

Authors: Yukio Ozaki, Shogo Tamura and Katsue Suzuki-Inoue

Cancer-associated thrombosis in Asia

Thrombosis is a common complication in cancer patients. Although the major inherited risk factors for thrombophilia are different between Asians and Caucasians, the main acquired risk factors that are associat...

Authors: Pantep Angchaisuksiri

Causes of venous thrombosis

Venous thrombosis which mainly manifests as deep vein thrombosis of the leg or pulmonary embolism occurs in 1 per 1000 per year. It occurs due to interacting genetic, environmental and behavioral risk factors....

Authors: F. R. Rosendaal

Management of venous thromboembolism: an update

Venous thromboembolism (VTE), which constitutes pulmonary embolism and deep vein thrombosis, is a common disorder associated with significant morbidity and mortality. Landmark trials have shown that direct ora...

Authors: Siavash Piran and Sam Schulman

Current animal models of hemophilia: the state of the art

Hemophilia is the most well-known hereditary bleeding disorder, with an incidence of one in every 5000 to 30,000 males worldwide. The disease is treated by infusion of protein products on demand and as prophyl...

Authors: Ching-Tzu Yen, Meng-Ni Fan, Yung-Li Yang, Sheng-Chieh Chou, I-Shing Yu and Shu-Wha Lin

MicroRNA in atherothromobosis: is it useful as a disease marker?

Atherosclerosis is one of the major causes of death. Data from animal experiments suggest that atherosclerosis involves an inflammatory process of the vascular wall under hyperlipidemia. Atherothrombosis can b...

Authors: Satoshi Fujii, Tomonori Sugiura, Yasuaki Dohi and Nobuyuki Ohte

Posttranslational forms of beta 2-glycoprotein I in the pathogenesis of the antiphospholipid syndrome

The antiphospholipid syndrome (APS) is an autoimmune disease characterised by a procoagulant state that predisposes to recurrent thrombosis and miscarriages. Two major discoveries have advanced our understandi...

Authors: Fatima El-Assaad, Steven A. Krilis and Bill Giannakopoulos

Clinical evaluation of thrombotic microangiopathy: identification of patients with suspected atypical hemolytic uremic syndrome

Atypical hemolytic uremic syndrome (aHUS) is a rare genetic disorder caused by defective complement regulation resulting in thrombotic microangiopathy (TMA). Patients can present as children or adults. The syn...

Authors: Yu-Min Shen

Anti-thrombotic agents derived from snake venom proteins

Snake venoms affect blood coagulation and platelet function in a complex manner. However, two classes of venom proteins, snaclecs and disintegrins have been shown to specifically target receptors including GPI...

Authors: Tur-Fu Huang, Chun-Chieh Hsu and Yu-Ju Kuo

DOACs – advances and limitations in real world

The group of new oral anticoagulants or NOACs, now termed direct oral anticoagulants or DOACs, with their favourable results from large scale phase III clinical trials, represent a major advancement and expand...

Authors: Lai Heng Lee

Proposal for new diagnostic criteria for DIC from the Japanese Society on Thrombosis and Hemostasis

Disseminated intravascular coagulation (DIC) is a serious disease that, in the presence of underlying disease, causes persistent, generalized, marked coagulation activation. Early treatment based on an appropr...

Authors: Hidesaku Asakura, Hoyu Takahashi, Toshimasa Uchiyama, Yutaka Eguchi, Kohji Okamoto, Kazuo Kawasugi, Seiji Madoiwa and Hideo Wada

Disseminated intravascular coagulation with the fibrinolytic phenotype predicts the outcome of patients with out-of-hospital cardiac arrest

We tested the hypothesis that disseminated intravascular coagulation (DIC) during the early phase of post-cardiopulmonary resuscitation (CPR) is associated with systemic inflammatory response syndrome (SIRS), ...

Authors: Takeshi Wada, Satoshi Gando, Yuichi Ono, Kunihiko Maekawa, Kenichi Katabami, Mineji Hayakawa and Atsushi Sawamura

A case report of asymptomatic aortic thrombosis incidentally detected by computed tomography in apparently healthy subject with a history of cancer surgery

Aortic thrombosis is a rare disease and only a few cases of the disease, especially associated with chemotherapy for malignant diseases and/or blood diseases, have been previously reported. Although Virchow’s ...

Authors: Tomonori Sugiura, Yasuaki Dohi, Sumiyo Yamashita, Shunsuke Murai and Nobuyuki Ohte

Extensive deep vein thrombosis treatment using fondaparinux and edoxaban: a case report

Factor Xa inhibitor is a key drug in the coagulation cascade. Parenteral anticoagulation using low molecular weight heparin or fondaparinux is the recommended form of treatment for most patients presenting wit...

Authors: Kazuhiro Shimizu, Takeshi Sasaki, Takanobu Tomaru and Hirofumi Noike

Vitamin K antagonist use: evidence of the difficulty of achieving and maintaining target INR range and subsequent consequences

Vitamin K antagonists (VKAs) are effective oral anticoagulants that are titrated to a narrow therapeutic international normalized ratio (INR) range. We reviewed published literature assessing the impact of INR...

Authors: Jeff R. Schein, C. Michael White, Winnie W. Nelson, Jeffrey Kluger, Elizabeth S. Mearns and Craig I. Coleman

Clinical benefit of graduated compression stockings for prevention of venous thromboembolism after total knee arthroplasty: post hoc analysis of a phase 3 clinical study of edoxaban

Guidelines from the Japanese Circulation Society recommend prophylaxis with anticoagulation plus intermittent pneumatic compression or graduated compression stockings (GCS) among patients at the highest risk f...

Authors: Takeshi Fuji, Satoru Fujita, Tetsuya Kimura, Kei Ibusuki, Kenji Abe, Shintaro Tachibana and Mashio Nakamura

Is it safe to withhold long-term anticoagulation therapy in patients with small pulmonary emboli diagnosed by SPECT scintigraphy?

The need for anticoagulation therapy (AC) in patients with subsegmental pulmonary embolism (SSPE) diagnosed by computed tomography of the pulmonary arteries (CTPA) has been questioned, as these patients run lo...

Authors: R. Ghazvinian, A. Gottsäter and J. Elf

Erratum to: ‘Oral rivaroxaban for Japanese patients with symptomatic venous thromboembolism – the J-EINSTEIN DVT and PE program’

Authors: Norikazu Yamada, Atsushi Hirayama, Hideaki Maeda, Satoru Sakagami, Hiroo Shikata, Martin H. Prins, Anthonie W. A. Lensing, Masaharu Kato, Junichi Onuma, Yuki Miyamoto, Kazuma Iekushi and Mariko Kajikawa

Evaluation of the chromogenic anti-factor IIa assay to assess dabigatran exposure in geriatric patients with atrial fibrillation in an outpatient setting

Dabigatran etexilate may be underutilized in geriatric patients because of inadequate clinical experience in individuals with severe renal impairment and post-marketing reports of bleeding events. Assessing th...

Authors: Luigi Brunetti, Betty Sanchez-Catanese, Leonid Kagan, Xia Wen, Min Liu, Brian Buckley, James P. Luyendyk and Lauren M. Aleksunes

Giant right ventricular outflow tract thrombus in hereditary spherocytosis: a case report

In hereditary spherocytosis with severe anemia, splenectomy is a recommended treatment. However, the spleen carries an important role both in immune function and coagulation. The increased risk of bacterial in...

Authors: Cedric Davidsen, Terje Hjalmar Larsen, Eva Gerdts and Mai Tone Lønnebakken

Dabigatran etexilate for thromboprophylaxis in over 5000 hip or knee replacement patients in a real-world clinical setting

Thromboprophylaxis is recommended for patients undergoing total hip or total knee replacement (THR, TKR). An international, open-label, prospective, observational, single-arm study in a routine clinical settin...

Authors: Nadia Rosencher, Charles M. Samama, Martin Feuring, Martina Brueckmann, Eva Kleine, Andreas Clemens and Simon Frostick

External validation of prognostic rules for early post-pulmonary embolism mortality: assessment of a claims-based and three clinical-based approaches

Studies show the In-hospital Mortality for Pulmonary embolism using Claims daTa (IMPACT) rule can accurately identify pulmonary embolism (PE) patients at low-risk of early mortality in a retrospective setting ...

Authors: Erin R. Weeda, Christine G. Kohn, Gregory J. Fermann, W. Frank Peacock, Christopher Tanner, Daniel McGrath, Concetta Crivera, Jeff R. Schein and Craig I. Coleman

Recurrent venous thrombosis in an adequately anticoagulated patient with pemphigus vulgaris

Several autoimmune skin disorders are characterised by an increased risk of thrombosis, with bollous pemphigoid carrying a higher risk than pemphigus vulgaris (PV). We describe the case of a middle aged gentle...

Authors: Paul R. J. Ames, Maria Graf and Fabrizio Gentile

Reviewer acknowledgment 2015

The Editors of Thrombosis Journal would like to thank all of our reviewers who have contributed to the journal in Volume 13 (2015) and whose valuable support is fundamental to its success.

Authors: Hugo ten Cate and Yukio Ozaki

Incidence of venous thromboembolism in France: a retrospective analysis of a national insurance claims database

Data estimating the annual incidence of venous thromboembolism (VTE) in France, taking into account both hospital and community settings, are very lacking.

Authors: Stéphane Bouée, Corinne Emery, Adeline Samson, Julie Gourmelen, Cécile Bailly and François-Emery Cotté

Venous thromboembolism and persistent pulmonary hypertension in cancer patients: a cross-sectional study

Cancer patients are at increased risk for venous thromboembolism (VTE).

Authors: Siegfried Wieshammer, Jens Dreyhaupt, Dirk Müller, Felix Momm and Andreas Jakob

Analysis of the potential of cancer cell lines to release tissue factor-containing microvesicles: correlation with tissue factor and PAR2 expression

Despite the association of cancer-derived circulating tissue factor (TF)-containing microvesicles and hypercoagulable state, correlations with the incidence of thrombosis remain unclear.

Authors: Camille Ettelaie, Mary EW Collier, Sophie Featherby, Naima E. Benelhaj, John Greenman and Anthony Maraveyas

Validation of a modified thromboelastometry approach to detect changes in fibrinolytic activity

Thus far, validated whole blood assays used in in vitro fibrinolysis experiments using thromboelastometry (ROTEM) are lacking or have yet to be tested in humans.

Authors: Gerhardus J. A. J. M. Kuiper, Marie-Claire F. Kleinegris, René van Oerle, Henri M. H. Spronk, Marcus D. Lancé, Hugo ten Cate and Yvonne M. C. Henskens

Original approach for thrombolytic therapy in patients with Ilio-femoral deep vein thrombosis : 2 years follow-up

The aim of the study was to discuss the results of catheter-directed thrombolysis and complementary procedures to treat acute iliofemoral deep vein thrombosis (DVT) evaluating the safety and effectivness of an...

Authors: Leslie Fiengo, Federico Bucci, Elias Khalil and Bruno Salvati

Global Thrombosis Test – a possible monitoring system for the effects and safety of dabigatran

Dabigatran is an alternative to warfarin (WF) for the thromboprophylaxis of stroke in patients with non-valvular atrial fibrillation (NVAF). The advantage of dabigatran over WF is that monitoring is not requir...

Authors: Kazunori Otsui, Diana A. Gorog, Junichiro Yamamoto, Takayuki Yoshioka, Sachiyo Iwata, Atsushi Suzuki, Toru Ozawa, Asumi Takei and Nobutaka Inoue

Aspirin for primary prevention of cardiovascular disease

Although aspirin has a well-established role in preventing adverse events in patients with known cardiovascular disease (CVD), its benefit in patients without a history of CVD remains under scrutiny. Current d...

Authors: Jobert Richie N. Nansseu and Jean Jacques N. Noubiap

Safety and efficacy of switching from low molecular weight heparin to dabigatran in patients undergoing elective total hip or knee replacement surgery

The aim of this study was to assess the safety and efficacy of switching therapy from low molecular weight heparin (LMWH; enoxaparin) to dabigatran for prevention of venous thromboembolic events (VTE) in patie...

Authors: Christian Wurnig, Andreas Clemens, Helmuth Rauscher, Eva Kleine, Martin Feuring, Reinhard Windhager and Josef Grohs

Oral dabigatran etexilate versus enoxaparin for venous thromboembolism prevention after total hip arthroplasty: pooled analysis of two phase 3 randomized trials

Two phase 3 trials compared 28–35 days of treatment with oral dabigatran 220 mg or 150 mg (RE-NOVATE) or 220 mg (RE-NOVATE II) once daily with subcutaneous enoxaparin 40 mg once daily for prevention of venous ...

Authors: Bengt I. Eriksson, Ola E. Dahl, Nadia Rosencher, Andreas Clemens, Stefan Hantel, Martin Feuring, Jörg Kreuzer, Michael Huo and Richard J. Friedman

Genetic risk factors in patients with deep venous thrombosis, a retrospective case control study on Iranian population

Venous thromboembolism (VTE) could be manifested as deep venous thrombosis (DVT) or pulmonary embolism (PE). DVT is usually the more common manifestation and is usually formation of a thrombus in the deep vein...

Authors: Soudabeh Hosseini, Ebrahim Kalantar, Maryam Sadat Hosseini, Shadi Tabibian, Morteza Shamsizadeh and Akbar Dorgalaleh

The management of acute venous thromboembolism in clinical practice – study rationale and protocol of the European PREFER in VTE Registry

Venous thromboembolism (VTE) is a major health problem, with over one million events every year in Europe. However, there is a paucity of data on the current management in real life, including factors influenc...

Authors: Giancarlo Agnelli, Anselm K. Gitt, Rupert Bauersachs, Eva-Maria Fronk, Petra Laeis, Patrick Mismetti, Manuel Monreal, Stefan N. Willich, Wolf-Peter Wolf and Alexander T. Cohen

Family history of venous thromboembolism is a risk factor for venous thromboembolism in combined oral contraceptive users: a nationwide case-control study

The aim was to assess the risk of venous thromboembolism (VTE) associated with use of combined oral contraceptives (COCs) in women with a family history of VTE.

Authors: Bengt Zöller, Henrik Ohlsson, Jan Sundquist and Kristina Sundquist

Low dose compared to variable dose Warfarin and to Fondaparinux as prophylaxis for thromboembolism after elective hip or knee replacement surgery; a randomized, prospective study

Deep vein thrombosis (DVT) and pulmonary emboli (PE), known together as venous thromboembolic (VTE) disease remain major complications following elective hip and knee surgery. This study compares three chemopr...

Authors: Murray M. Bern, Diane Hazel, Elizabeth Deeran, John R. Richmond, Daniel M. Ward, Damon J. Spitz, David A. Mattingly, James V. Bono, Ronna H. Berezin, Laura Hou, Gerald B. Miley and Benjamin E. Bierbaum

Should lifelong anticoagulation for unprovoked venous thromboembolism be revisited?

Venous thromboembolism [VTE] is a common medical condition that has significant morbidity and mortality. Although stringent guidelines recommend lifelong anticoagulation for patients with unprovoked VTE, the o...

Authors: Otto Moodley and Hadi Goubran

Markers of thrombin generation are associated with myocardial necrosis and left ventricular impairment in patients with ST-elevation myocardial infarction

Platelet activation, thrombin generation and fibrin formation play important roles in intracoronary thrombus formation, which may lead to acute myocardial infarction.

Authors: C. H. Hansen, V. Ritschel, S. Halvorsen, G. Ø. Andersen, R. Bjørnerheim, J. Eritsland, H. Arnesen and I. Seljeflot

Unsuccessful percutaneous mechanical thrombectomy in fibrin-rich high-risk pulmonary thromboembolism

We report a case of unsuccessful percutaneous mechanical thrombectomy in treatment of a high-risk pulmonary embolism (PE). Pulmonary thromboemboli are commonly expected as a homogenous mass, rich with red bloo...

Authors: Jernej Vidmar, Igor Serša, Eduard Kralj and Peter Popovič