Research and Practice in Thrombosis and Haemostasis

Great white sighting: a case of heparin-induced thrombosis with thrombocytosis
Levy J, De Sa H, Loss L, VanSandt M, Patel R and Sendowski M
Heparin-induced thrombocytopenia (HIT) is an immune-mediated adverse response to heparin therapy, characterized by decreased platelet count and increased risk of thrombosis. HIT, without the tell-tale sign of thrombocytopenia, has rarely been described.
How to treat patients with bleeding disorder of unknown cause?
Mussert C, Monard A and Heubel-Moenen F
Multiple myeloma: retrospective assessment of routine thromboprophylaxis and utility of thrombotic risk scores
Fernandez-Vargas OE, Amezcua I, Cabello B, Quintana Martinez A, Espinoza R and Cesarman-Maus G
The high risk of venous thromboembolism (VTE) in multiple myeloma (MM) warrants primary thromboprophylaxis for most patients. Myeloma-specific thrombotic risk scores (TRSs), such as IMPEDE-VTE, SAVED, and PRISM, were developed to improve risk assessment and guide antithrombotic strategies. Their performance is variable and has not yet been tested in Latin America.
Comparable hemostatic capacity of blood taken from the portal vein compared with systemic blood in patients with cirrhosis
Blasi A, Calvo A, Mellado R, Torrente MA, Turon F, Garcia-Pagan JC, Hernandez-Gea V, Tassies D, Reverter JC and Lisman T
Clinical features and treatment of 70 children with lupus anticoagulant-hypoprothrombinemia syndrome: a retrospective study from a single center in China
Tian D, Zhang J, Lou J, Chen X, Liang J, Xu X, Gao H, Nie W, Ye Q and Shen H
Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS) is a rare acquired bleeding disorder characterized by the presence of lupus anticoagulant (LA) and acquired hypoprothrombinemia.
Mechanistic insight into multiple antibody binding to ADAMTS13 in immune thrombotic thrombocytopenic purpura
Halkidis K, Meng C, Pillai VG, Shay M, Liu S and Zheng XL
Antibody-mediated inhibition of von Willebrand factor (VWF) cleavage by ADAMTS-13 results in immune thrombotic thrombocytopenic purpura (iTTP). However, the effects of multiple antibody binding to ADAMTS-13 are not fully understood.
Safety and efficacy of direct oral anticoagulants in chronic kidney disease: a meta-analysis
Tham D, Zhao L, Yu W, Kherani J, Kou R, Li A, Li PY, Eshaghpour A and Crowther MA
Direct oral anticoagulants (DOACs) have emerged as the first-line therapy for venous thromboembolism and stroke prophylaxis in atrial fibrillation. As DOACs are partially excreted renally, their safety in patients with chronic kidney disease (CKD) is unclear.
Thrombin generation to evaluate the complex hemostatic balance of hemophilia A plasma containing direct oral anticoagulant and supplemented by factor VIII
Lamoine S, Jury V, Fourneyron V, Douxfils J, Teissandier D, Talon L, Sinegre T and Lebreton A
The incidence of cardiovascular diseases is increasing in persons with hemophilia A (HA). Therefore, anticoagulant therapy based on direct oral anticoagulants (DOACs) may be needed, despite the bleeding risk. In case of surgery or bleeding, such patients may be concomitantly treated with emicizumab (routine prophylaxis), factor (F)VIII products, and DOAC. Their concomitant presence constitutes a hemostatic challenge. Recent international guidelines stated that data are scarce on the hemostatic balance of plasma samples from patients with HA receiving emicizumab and DOAC.
Reported particles are not blood clots, so anticoagulant drugs are not a plausible treatment
Fox T, Hunt B, Carson A, Scandrett K, Smith GD, Garner P and Kuehn R
Loss of endogenous Nox2-NADPH oxidase does not prevent age-induced platelet activation and arterial thrombosis in mice
Ahmed A, Patil G, Sonkar VK, Jensen M, Streeter J and Dayal S
Reactive oxygen species are known to contribute to platelet hyperactivation and thrombosis during aging; however, the mechanistic contribution of the specific oxidative pathway remains elusive.
Retrospective analysis of hemophilia B in Turkey: identifying main characteristics and treatment options
Zülfikar B, Koç B, Şahin F, Şaşmaz Hİ, Kavaklı K, Balkan C, Antmen AB, Akbayram S, Güvenç B, Okan V, Türkkan E, Albayrak C, Albayrak D, Sarper N, Celkan TT, Ayyıldız O, Aksu S, Patıroğlu T, Şalcıoğlu Z, Güneş AM, Torun YA, Çalışkan Ü, Tokgöz H, Ay Y, Özdemir GN, Sönmez M, Ünal E, Öner AF, Güler N, Küpesiz OA, Ören H, Karaman S, Ünüvar A, Dağlı M, Demir AM, Söker M, Alioğlu B, Kaya Z, Ayhan AC, Bıçakçı Z, Aral YZ and Ar MC
Hemophilia B (HB), an X-linked recessive inherited bleeding disorder, exhibits a high prevalence among males.
Balancing safety and efficacy of low-molecular-weight heparins in neonates: a systematic review
Verweij M, van Weissenbruch MM and Klaassen ILM
The rising incidence of venous thromboembolism (VTE) in neonates has led to increased use of low-molecular-weight heparins (LMWHs), but optimal dosages remain uncertain. A serious adverse effect of LMWHs is major bleeding. Given the vulnerability of neonates to major bleeding, we aimed to review therapeutic and prophylactic LMWH dosages to achieve target anti-factor Xa ranges of 0.5 and 1.0 U/mL and 0.1 and 0.4 U/mL, respectively. Our secondary aim was to assess the safety and efficacy of LMWHs in neonates. A systematic review of all published studies between 1996 and 2023 that pertained to the dosing, safety, or efficacy of LMWH in preterm and term neonates. Studies were identified through the Medline database. Data on LMWH dosages, bleeding events, resolution and recurrence, and anti-factor Xa levels were analyzed. A total of 38 studies involving 1145 neonates were included. To achieve a therapeutic or prophylactic target range, weight-adjusted initial dosages of LMWH had to be increased by 21% particularly in premature neonates. During therapeutic therapy, major bleeding occurred in 4.1% and minor bleeding in 7.1%. During prophylactic therapy, 11.4% experienced major bleeding and 17.1% minor bleeding. With therapeutic dosages, 55.8% achieved complete VTE resolution. Additionally, 68.5% of neonates initially failed to achieve therapeutic anti-factor Xa levels, persisting in 29.4% despite dose adjustments. A higher initial therapeutic dosage of LMWHs may be needed in neonates. In addition, the patient's gestational age must be considered in the dosing strategy to optimize outcomes. Although this must be weighed against bleeding risk at an individual level.
Extracellular vesicle tissue factor and tissue factor pathway inhibitor are independent discriminators of sepsis-induced coagulopathy
Tobiasch AK, Lehner GF, Feistritzer C, Peer A, Zassler B, Neumair VM, Klein SJ and Joannidis M
Sepsis-induced disseminated intravascular coagulopathy (DIC) remains a challenging clinical entity associated with significant morbidity and mortality. Endothelial injury or activation and extracellular vesicles (EV) are postulated as important determinants of DIC.
Bleeding and quality of life in people with Glanzmann thrombasthenia-insights from the Glanzmann's 360 study
Khair K, Fletcher S, Boyton M and Holland M
Glanzmann thrombasthenia (GT) is a rare platelet function disorder that results in severe bleeding. We assessed clinical symptoms and psychological parameters to identify the unmet needs associated with GT.
A cluster of pediatric vaccine-induced immune thrombotic thrombocytopenia-like cases with thrombosis and thrombocytopenia following respiratory infections-case series
Dimopoulou D, Mentesidou L, Dettoraki A, Karastathi C, Berikopoulou M, Katsouli P, Anastasopoulou I, Stamatakis IG, Bachou T, Tzifi F, Michalopoulou A, Messaritaki A, Spoulou V and Pergantou H
Adenoviral vector COVID-19 vaccine-induced immune thrombotic thrombocytopenia (VITT) is a heparin-independent platelet-activating disorder. An increasing number of VITT-like disorders without previous vaccination are being identified.
Functional characterization of a nanobody-based glycoprotein VI-specific platelet agonist
Zivkovic M, Pols-van Veen E, van der Vegte V, Sebastian SAE, de Moor AS, Korporaal SJA, Schutgens REG, Urbanus RT, and
Glycoprotein (GP)VI is a platelet-specific collagen receptor required for platelet activation during hemostasis. Platelet reactivity toward collagen is routinely assessed during diagnostic workup of platelet disorders. GPVI can be activated by inducing receptor clustering with suspensions of fibrillar collagen or synthetic cross-linked collagen-related peptide (CRP-XL). However, these suspensions are poorly standardized or difficult to produce. Nanobodies are small recombinant camelid-derived heavy-chain antibody variable regions. They are highly stable, specific, and ideal candidates for developing a stable GPVI agonist for diagnostic assays.
Prediction of the chance of successful immune tolerance induction in persons with severe hemophilia A and inhibitors: a clinical prediction model
Oomen I, Abdi A, Camelo RM, Callado FMRA, Carvalho LEM, Calcaterra IL, Carcao M, Castaman G, Eikenboom JCJ, Fischer K, Franco VKB, Heymans MW, Leebeek FWG, Lillicrap D, Lorenzato CS, Mancuso ME, Matino D, Di Minno MND, Mohseny AB, Oldenburg J, Rezende SM, Rivard GE, Rydz N, Schols SEM, Voorberg J, Fijnvandraat K, Gouw SC and
Inhibitor eradication to restore factor (F)VIII efficacy is the treatment goal for persons with severe hemophilia A (HA) and inhibitors. Immune tolerance induction (ITI) is demanding and successful in about 70% of people. Until now, it has remained difficult to quantify the probability of ITI success or failure, complicating the decision to initiate or not initiate ITI. Estimating the individual chance of ITI success allows clinicians, patients, and their families to support shared decision-making.
Periprocedural hemostatic prophylaxis and outcomes in bleeding disorder of unknown cause
Berkowitz C, Ma A, Miller V, Goraya S, Kirkland K, Grabell J, Key NS and James PD
Bleeding disorder of unknown cause (BDUC) is a diagnostic category encompassing patients with a clear bleeding phenotype but without identifiable abnormality on hemostatic testing. The optimal management of hemostasis in BDUC patients prior to invasive procedures and childbirth is uncertain.
Hospital-acquired venous thromboembolism among critically ill children with diabetic ketoacidosis: a multicenter, retrospective cohort study
DeMayo KM, Havlicek EE, Betensky M, Goldenberg NA and Sochet AA
Critically ill children and young adults with diabetic ketoacidosis are thought to be in a prothrombotic state. However, the rate of hospital-acquired venous thromboembolism and associated risk factors in this population have not been identified.
Distinguishing hyperfibrinolysis from enhanced-fibrinolytic-type disseminated intravascular coagulation
Yamada S, Miyamoto T and Asakura H
Clinical characteristics, treatment, and outcomes of provoked acute cerebral sinovenous thrombosis in patients <21 years old: findings from the Kids-DOTT Multinational Trial
Woods GM, Miller A, Mosha M, Male C, Verma A, Kucine N, Sabapathy C, Beg K, Ahuja S, Raybagkar D, Hege K, Lo C, Bhat R, Abshire T and Goldenberg NA
Prospective multicenter data on the treatment and outcomes of children with cerebral sinovenous thrombosis (CSVT) are limited. We aimed to describe the clinical characteristics, treatment strategies, and outcomes of patients with a first-episode of provoked acute CSVT enrolled in the Kids-DOTT trial and compare these features with those of participants with non-CSVT venous thromboembolism (VTE).