Hamostaseologie最新文献

Chronic high-altitude hypoxia is associated with reduced platelet count, but it is unclear whether the decrease in platelet count is due to impaired production or increased clearance. This study examines how hypoxia affects platelet production and apoptosis and elucidates the impact of glycoprotein Ibα-von Willebrand factor interaction on platelets in rats using a hypobaric hypoxia chamber. The results showed that the number of megakaryocytes increased under hypoxia; however, the levels of differentiation and polyploidy decreased, while those of apoptosis increased. Platelet production did not reduce according to the reticulated platelet percentage, while platelet apoptosis enhanced; these results suggest that increased platelet clearance was the main reason behind platelet reduction. Our previous microarray results indicated that glycoprotein Ibα (GPIbα) expression increased under hypoxia, which was a protein involved in platelet clearance; therefore, we examined the interaction of platelet GPIbα with the von Willebrand factor (vWF) both in vivo and in vitro to explore the effect of this process on platelets and whether it is related to platelet apoptosis. Under hypoxia, the stronger interaction between GPIbα and vWF promoted platelet apoptosis; inhibiting this interaction reduced platelet apoptosis and increased platelet counts. Platelet reduction is associated with apoptosis induced by the interaction between GPIbα and vWF.

High neutrophil-to-lymphocyte ratio (NLR) and platelet-to-lymphocyte ratio (PLR) are markers of subclinical inflammation and have been associated with prognosis and mortality in many diseases. In this study, we evaluated the comparative value of NLR and PLR in identifying high mortality risk in patients hospitalized with acute pulmonary thromboembolism (PTE), and their relationship with the anatomical burden of thrombus.Patients who were followed up due to PTE were evaluated retrospectively. NLR and PLR were calculated from complete blood counts. The thrombus burden was assessed by the Qanadli score; based on the patients' archival computed tomography angiography images. Mortality prediction was based on an algorithm using the Pulmonary Embolism Severity Index, echocardiographic findings, and troponin levels.Three hundred-two PTE patients were included in the study. Median NLR, PLR, and Qanadli score values were higher in nonsurvivors, with NLR (8.4 [2.2-18.9]) vs. (3.1 [0.4-13.1]), PLR (317 [87.6-525.3]) vs. (124.4 [30-476.3]), and Qanadli scores (21 [3-26]) vs. (9 [1-28]), respectively (p < 0.001). We showed that setting a threshold value of >4.45 for NLR and >151.59 for PLR significantly predicts the high mortality-risk group. In the receiver operating characteristic analysis, when the threshold value for the Qanadli score distinguishing between low-risk and high-risk disease was set at 15.5, the sensitivity was calculated as 98.8% and the specificity was 94.9% (p = 0.001).This study showed that NLR, PLR, and Qanadli scores can provide essential contributions to the clinician's determination of the anatomical burden of thrombus and disease severity in PTE patients.

Introduction:  Treatment options for patients with hemophilia (PWH) have changed substantially in the last years. This study aimed to compare hemophilia treatment in the eastern part of Germany in 2021 with data from 2015.

Methods:  Substitution diaries and patient records of PWH from 2021 were collected in 13 hemophilia centers from the "Kompetenznetz Hämorrhagische Diathese Ost" (KHDO) and compared with 2015.

Results:  A total of 130 children and 357 adults, 411 hemophilia A (HA) and 76 hemophilia B (HB), were included in 2021, and 359 were already analyzed in 2015. In 2021, 97.8% of children and 95.7% of adults with severe hemophilia had prophylaxis compared with 98.8 and 80.2% in 2015. Plasma-derived concentrates were used in 25.6%, recombinant in 30.2%, extended half-life (EHL) factor concentrates in 24.4%, and emicizumab in 19.8% of the children with severe HA (sHA). In adults with sHA, plasma-derived, recombinant concentrates, EHL, and emicizumab were used in 21.0, 33.2, 31.2, and 14.2%, respectively. All children and 93.3% of the adults with severe HB (sHB) were on EHL. Median annual factor consumption per body weight increased in adults with sHA, remained stable in children with sHA and adults with sHB, and decreased in children with sHB between 2015 and 2021. Annualized bleeding rate (ABR) decreased in children with sHB and sHA.

Conclusion:  The use of EHL and emicizumab has changed hemophilia treatment. About 50% of the sHA patients switched to EHL or emicizumab and almost all sHB patients to EHL. More adults with sHA received prophylaxis and ABR decreased in children.

Thrombocytopenia at admission predicts mortality in multiple myeloma (MM) and might link to disease progression. Although thrombocytopenia is known to be associated with MM, a possible thrombopathy is clinically less known. We conducted a case-control study comparing platelet responses of MM patients to controls via flow cytometry, integrin αIIbβ3 activation and P-selectin exposure, and a bioluminescent assay, ATP release. No difference was found at baseline, but upon platelet stimulation, MM patients had decreased αIIbβ3 activation, partly impaired P-selectin exposure, and reduced δ-granule (ATP) secretion. Aspirin treatment in patients did not account for these diminished platelet responses. In total, 29% of patients had thrombocytopenia, while 60% had decreased αIIbβ3 activation and 67% had reduced platelet secretion capacity. Importantly, as secretion capacity was corrected for platelet count, granule release per platelet was reduced in patients versus controls. Of 6 patients with thrombocytopenia 4 displayed a thrombopathy, while for 15 patients with normal count, 64% had reduced αIIbβ3 activation and 73% had reduced platelet secretion capacity. Of all patients, 10% had thrombocytopenia combined with reduced αIIbβ3 activation plus low secretion capacity (one patient showed no qualitative or quantitative platelet defect). Our data suggest that beyond the known thrombocytopenia, MM patients also have reduced platelet function, which could reflect impaired platelet vitality. Combined measurement of platelet count and function, especially secretion capacity, gives a more comprehensive view of platelet phenotype than count alone. Large prospective follow-up studies are needed to confirm the importance of the acquired platelet secretion defect on the prognosis of MM patients.

The 2025 Annual Congress of the Society of Thrombosis and Haemostasis Research (GTH) takes its inspiration from ARTE-Advances, Research, Technology, and Education in the field of thrombosis and hemostasis. The numerous scientific contributions of the congress highlight the most recent progresses in this field, and reveal the profound connection between the rigor of science and the beauty of human creativity. ARTE, the Italian word for "art," refers to the deep synergy existing between analytical precision and imaginative expression, which is vividly reflected in this year's contributions to our themed congress issue.

Combining diagnostics and research in academic laboratories faces challenges and bears great opportunities. In this short review, we describe the objectives of diagnostic and research laboratories dealing with thrombosis and hemostasis questions. We give an overview of specific goals for diagnostic and research laboratories and explain the synergies and tasks which need to be managed in an interdisciplinary team.

Hereditary bleeding disorders encompass a range of hemostasis defects that impair the blood coagulation process. Although these disorders affect both men and women, research and clinical management have historically been predominantly focused on male patients, particularly those with hemophilia. Consequently, the impact of these disorders on women has been undervalued and frequently overlooked. The intricate relationship between a woman's tendency to bleed and the various gynecological and obstetric processes gives rise to distinctive health challenges for women with hereditary bleeding disorders. Heavy menstrual bleeding (HMB), excessive bleeding during miscarriages, postpartum hemorrhage, and hemorrhagic ovarian cysts represent some of the most common complications. Despite the high prevalence and significant impact of these symptoms, many women experience delays in diagnosis and treatment, which in turn may result in iron-deficiency anemia, anxiety, influence on reproductive decisions, and a decreased quality of life. This review aims to summarize the distinctive characteristics of hereditary bleeding disorders in women, emphasizing the clinical challenges and hormonal management strategies for HMB.

Chimeric antigen receptor (CAR) T cell therapy has revolutionized cancer immunotherapy, particularly for hematological malignancies. This personalized approach is based on genetically engineering T cells derived from the patient to target antigens expressed-among others-on malignant cells. Nowadays they offer new hope where conventional therapies, such as chemotherapy and radiation, have often failed. Since the first FDA approval in 2017, CAR T cell therapy has rapidly expanded, proving highly effective against previously refractory diseases with otherwise a dismal outcome. Despite its promise, CAR T cell therapy continues to face significant challenges, including complex manufacturing, the management of toxicities, resistance mechanisms that impact long-term efficacy, and limited access as well as high costs, which continue to shape ongoing research and clinical applications. This review aims to provide an overview of CAR T cell therapy, including its fundamental concepts, clinical applications, current challenges, and future directions in hematological malignancies.