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FEIBA® (factor eight inhibitor by-passing activity) is used to achieve haemostasis in haemophiliacs with inhibitor. The aim of this study was to evaluate efficacy and consumption of the product in treatment of haemorrhages in haemophiliacs with factor VIII inhibitor, and determine factors that can influence the results of treatment. We used data from our haemophilia centre from years 2000–2008. Six haemophiliacs with factor VIII inhibitor were treated on demand with FEIBA® for 61 bleeding episodes (45 haemarthroses, six muscle bleeds, six other sites bleeds and four multiple sites bleeds). The median cumulative dose of FEIBA® per bleeding episode was 205 U kg−1. Bleeding was stopped in 96.7% (59 of 61) of events but re-bleeding occurred in 3 events (4.9%) within 48 h after cessation of bleeding. In home treatment (20 of 61) bleeding stopped in 90% (18 of 20) without recurrence and the median consumption per event was reduced to 153 U kg−1. Without the use of home treatment the median consumption was 250 U kg−1 per event and bleeding ceased definitely in 92.7% (38 of 41) of cases. The cumulative dose of FEIBA® was lower for three episodes with re-bleeding: median 96 U kg−1 but not in the two cases of ineffective treatment: 361 U kg−1. FEIBA® in management of bleeding episodes completely resolved the haemorrhage in 91.8% of events and in a further 4.9% if treatment was restarted. Using home treatment saved expenditure due to the lower cumulative dose needed for treatment of haemorrhage. [ABSTRACT FROM AUTHOR]

High-grade B-cell lymphomas (HGBCLs) are aggressive blood cancers with a severe disease course, especially when the central nervous system (CNS) is involved. Standard histological examination depends on tissue availability and is currently supplemented with molecular tests, as the status of MYC, BCL2, or BCL6 gene rearrangements is required for proper lymphoma classification. This case report demonstrates the relevance of cerebrospinal fluid (CSF) cell-free DNA testing by integrative next-generation sequencing (NGS) panel. The benefit of this approach resided in tumor genotyping alongside the proof of CNS progression despite MRI negativity, revealing a clonal relationship with the primary tumor lesion. In addition, our strategy allowed us to classify the tumor as DLBCL/HGBL-MYC/BCL2 entity. In clinical practice, such a minimally invasive approach provides a more sensitive tool than standard imaging and cell analyzing techniques, enabling more accurate disease monitoring and relapse prediction in particular cases., Competing Interests: Declarations. Ethical approval: Written informed consent, approved by the University Hospital Brno Ethics Committee (23-090621-EK), was obtained from the patient to publish any potentially identifiable data in this article. Consent for publication: Not applicable. Competing interests: The authors declare no competing interests., (© 2025. The Author(s).)

The clinical course of essential thrombocythemia (ET) is complicated with thrombosis which significantly impacts patients' mortality. Studies have identified JAK2V617F mutation as an independent risk factor for thrombosis. Circulating extracellular vesicles (EVs) were evaluated in several studies regarding myeloproliferative neoplasms and thrombosis as potential biomarkers. The present study investigates the relationship between JAK2V617F mutation and EVs levels in 119 ET patients. Our analyses revealed that JAK2V617F-positive patients are at a significantly increased risk of thrombosis within five years before the ET diagnosis (hazard ratio [95% CI]: 11.9 [1.7-83.7], P = 0.013), and that JAK2V617F mutation is an independent risk factor for thrombosis at ET diagnosis or during the follow-up (hazard ratio [95% CI]: 3.56 [1.47-8.62], P = 0.005). ET patients have higher levels of platelet-EVs, erythrocyte-EVs and procoagulant activity of EVs than the healthy population. Absolute and relative counts of platelet-EVs are increased in the presence of JAK2V617F mutation (P = 0.018, P = 0.024, respectively). In conclusion, our results support the role of JAK2V617F mutation in the pathogenesis of thrombosis in essential thrombocythemia through enhancing platelet activation.

Background/aim: This work aimed to prospectively evaluate the clinical significance of circulating microparticles (MPs) in relation to thrombotic risk factors and thrombotic complications in patients with BCR/ABL1-negative myeloproliferative neoplasms (MPN)., Patients and Methods: In a cohort of 206 patients with MPN, MPs' procoagulant activity was measured by the Zymuphen functional assay in 429 samples, while platelet- and erythrocyte-MPs were enumerated by flow cytometry in 558 samples., Results: MPN patients had higher MP levels than the control group. The levels of MPs were higher in male patients, smokers, and those who were older than 60 years, and in the presence of JAK2 V617F mutation, history of thrombosis, platelets >400×10 9 /l, hematocrit >45%, or leukocytes >10×10 9 /l. Cytoreductive treatment reduced MP levels, with anagrelide being associated with lower MP levels than hydroxyurea., Conclusion: The relationship with thrombotic risk factors indicates a possible role of MPs in the complex thrombotic mechanism, though cytoreductive treatment seems to affect this role through reducing MP levels., (Copyright © 2021 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Thrombosis is the most common complication in BCR-ABL1 negative myeloproliferative neoplasms (MPN) that significantly impacts patients' mortality. Generally, there is an agreement on risk factors that possibly contribute to the increased risk of thrombosis, including age, history of thrombosis, JAK2V617F mutation, and cardiovascular risk factors. This study retrospectively investigates MPN-related and patient-related variables in relation to the thrombosis occurrence in MPN. Our analyses show that JAK2V617F-mutated patients are at a significantly increased risk of thrombosis within five years before the MPN diagnosis point with a hazard ratio (HR) of 15.49 (p=0.006). In multivariate analyses, independent risk factors for thrombotic complications during the follow-up are history of thrombosis (HR=2.23, p=0.019), age over 60 years at diagnosis (HR=1.56, p=0.037), the presence of JAK2V617F mutation (HR=3.01, p=0.002), and tobacco smoking (HR=1.75, p=0.01). Our results support the multifactorial mechanism of thrombosis in MPN patients, which demands individual and complex management.

Microparticles are small membrane fragments with dimension between 0.1 and 1 μm, which are released during cell activation or apoptosis, exposing the phospholipid phosphatidylserine and membrane antigens typical for cellular origin. Philadelphia-negative myeloproliferative neoplasms (MPNs) are characterized by an increased risk of thrombosis. Data from literature suggest an association between thrombosis and the procoagulant activity of microparticles. Association between the procoagulant activity of microparticles and the incidence of thrombosis was assesed in a group of 126 patients with Philadelphia-negative MPNs. Measurement of microparticles procoagulant activity was performed using a functional assay, namely the Zymuphen MP-activity (Hyphen Biomed, Neuville-sur-oise, France). A total of 539 samples were analysed within this group of patients, regardless of patients' state of health. A significantly higher circulating microparticles procoagulant activity was found in MPN patients as compared with the control group (P < 0.001). A pathological level of procoagulant activity was observed more frequently in patients with polycythaemia vera (88%, P = 0.002) than groups of patients with essential thrombocythaemia (73.2%) and primary myelofibrosis (68.3%); the same result was confirmed in patients with a history of venous thrombosis in comparison with patients without thrombosis (84.7 vs. 73.2%, P = 0.029). Patients without cytoreductive treatment had a higher activity of microparticles (P = 0.010). Furthermore, presence of JAK2 V617F mutation was associated with an increased procoagulant activity (P = 0.007), as well as the higher JAK2 V617F allele burden (P = 0.001). Further prospective clinical studies will be necessary to evaluate the clinical relevance of microparticles in the prediction hypercoagulable state in these patients.

BCR/ABL-negative myeloproliferative neoplasms (MPNs) are considered to be acquired thrombophilic conditions. Persistently enhanced platelet activation has been described in polycythaemia vera and essential thrombocythaemia (ET), and shown to contribute to a higher risk of arterial and venous thrombotic complications. Recent studies have shown that mean platelet volume (MPV) and immature platelet fraction (IPF) can serve as useful markers of platelet activation and increased risk of thrombosis. The aim of the present study was to investigate the relationship between these parameters and thrombotic events in BCR/ABL-negative MPN. MPV values in patients with BCR/ABL-negative MPN were significantly higher than MPV values of healthy individuals (P < 0.001). No significant difference in MPV or IPF was observed between groups of patients with and without thrombotic complications (P = 0.441; P = 0.110); the difference in IPF values was close to the significance level for patients with ET (P = 0.073). Higher values of IPF were more frequently detected in patients with JAK2 V617F positivity (P = 0.030). These patients had higher MPV more frequently than others, and this difference was close to the significance level (P = 0.056). Further studies should validate the use of platelet parameters to identify patients at high risk.

Aim: The aim of this work was to retrospectively analyze patients with Philadelphia-negative myeloproliferative neoplasms through evaluation of frequency and characteristics of second malignancies (other than acute leukaemia and myelodysplastic syndrome)., Patients and Methods: Records of 172 patients were reviewed; an analysis was performed on data from 66 patients treated with hydroxyurea, 105 patients treated with other cytoreductive therapy, and 25 patients without treatment., Results: A higher occurrence of second malignancies was found in the group treated with hydroxyurea (7.6%; other cytoreduction: 1.2%; without therapy: 0). After a median follow-up of 89 months in the hydroxyurea group, 13 patients developed second cancer during hydroxyurea therapy, located on the skin (68.75%) and other sites (31.25%)., Conclusion: The incidence of second malignancies during hydroxyurea therapy in our cohort patient was significantly higher than the incidence of malignancies in the Czech population of corresponding age.