Hamostaseologie最新文献

The increasing age of patients with congenital hemophilia poses new challenges for clinicians. Not only the consequences of bleeding but also age-related diseases, especially cardiovascular disorders, are of great concern. Factors such as hypertension, diabetes, or hyperlipidemia further increase the cardiovascular risk in elderly patients with hemophilia. Preventive treatment of these cardiovascular diseases may therefore also be necessary in hemophilia patients. However, determining the optimal level of anticoagulation in patients with reduced levels of coagulation factors is often difficult and requires an individualized approach. Given the absence of substantial evidence from large clinical trials, clinicians rely on case reports and expert opinions to guide the therapy. This case report details the individual risk-benefit assessment and management of a 57-year-old hemophilia A patient with atrial fibrillation.

Cancer-associated thrombosis (CAT) remains a critical concern in hematology and oncology, contributing significantly to morbidity and mortality. The interplay between malignancy and hemostasis has been extensively studied, yet it continues to present clinical challenges and opportunities for advancement in prevention, treatment, and management. This special issue of Haemostaseologie-Progress in Haemostasis-Disorders of Thrombosis and Hemostasis in Cancer brings together seven review articles that explore diverse aspects of CAT, shedding light on current practices and emerging trends.

Patients with thrombosis at an unusual site will need to be explored for rare causes of thrombosis. Two of these rare causes include myeloproliferative neoplasms (MPNs) and paroxysmal nocturnal hemoglobinuria (PNH). It is important not to overlook these causes, since they require specific management, in addition to antithrombotic treatment (anticoagulants, antiplatelet agents). Unusual sites of venous thrombosis include upper extremity veins, splanchnic veins, cerebral veins, and retinal veins, and unusual sites of arterial thrombosis include renal, adrenal, splenic and mesenteric arteries, and intracardiac and aortal locations. Suspicion for MPN and PNH should be raised if there are concomitant abnormalities, such as elevated or decreased blood cell counts or splenomegaly. Diagnosis of MPN and PNH should include JAK2V617F mutational screening as well as flow cytometric assessment of GPI-anchored proteins in the peripheral blood, respectively. Specific treatments for MPN may include phlebotomy or cytoreductive drugs such as hydroxyurea, anagrelide, pegylated interferon-alpha, or Janus kinase inhibitors. Drugs used for PNH treatment include terminal complement inhibitors, such as eculizumab and ravulizumab, as well as proximally acting inhibitors such as pegcetacoplan or iptacopan. Patients with MPN and PNH are at high risk for thrombosis during their entire lifetime and should thus be followed by specialists experienced in the care of these diseases.

Immune checkpoint inhibitors (ICIs) and chimeric antigen receptor (CAR) T cells are novel therapeutic strategies that enhance anticancer immunity by activating or engineering cancer-targeting T cells. The resulting hyperinflammation carries several side effects, ranging from autoimmune-like symptoms to cytokine release syndrome (CRS), with potentially severe consequences. Recent findings indicate that ICIs increase the risk of venous and arterial thromboembolic adverse events. Patients with prior VTE might be at higher risk of developing new events under ICI while other risk factors vary across studies. So far, data on CAR T-linked coagulopathies are limited. Hypofibrinogenemia in the presence of CRS is the most commonly observed dysregulation of hemostatic parameters. A rare but particularly severe adverse event is the development of disseminated intravascular coagulation activation, which can occur in the setting of CRS and may be linked to immune effector cell-associated hemophagocytic lymphohistiocytosis. While the increasing number of studies on thromboembolic complications and coagulation alterations under ICIs and CAR T therapies are concerning, these results might be influenced by the retrospective study design and the heterogeneous patient populations. Importantly, numerous promising new T cell-based immunotherapies are currently under investigation for various cancers and are expected to become very prominent therapy options in the near future. Therefore, coagulopathies and thrombosis under T cell-directed immuno- and anti-cancer therapies is important. Our review provides an overview of the current understanding of ICI- and CAR T-associated thromboembolism. We discuss pathogenic mechanisms of inflammation-associated coagulation activation and explore potential biomarkers for VTE.

The hemostatic system and cancer display a tight interconnection, and hemostatic imbalance frequently occurs in patients with cancer. While extensive knowledge about thrombotic risk has been generated, less is known about bleeding risk and associated risk factors. However, bleeding risk is of high significance as patients with cancer frequently receive therapeutic anticoagulation for various indications and/or are candidates for primary thromboprophylaxis. The risk of bleeding in patients with cancer is variable and difficult to assess in clinical practice. Certain clinical settings such as hospitalization, specific underlying risk factors (e.g., tumor type), and medications (e.g., anticoagulation) can contribute to the individual bleeding risk of a patient with cancer. In addition, some dynamic factors such as platelet count or kidney function have an impact. Particularly, data on baseline risk of bleeding are lacking to allow for risk assessment in cancer patients without anticoagulation. In contrast, risk assessment models for the prediction of bleeding events in cancer patients receiving anticoagulation have been developed; however, these have yet to be validated. The recognition of the importance of bleeding risk in cancer patients is growing, leading to an increasing number of studies investigating and reporting bleeding complications. As study designs and reporting of bleeding events vary, it is challenging to offer a clear synthesis of evidence. In this narrative review, we provide an overview of currently available data about incidence, risk factors, and clinical impact of bleeding events in patients with cancer, and critically review risk assessment models for bleeding in cancer patients during anticoagulant therapy.

Over the past two decades, the incidence of cancer-associated thrombosis (CAT) has increased. It is nowadays a common and often serious complication among patients with cancer. Although medical thromboprophylaxis is recommended for most surgical and nonsurgical cancer patients, it has been infrequently used in ambulatory patients with cancer because of the burden of treatment and concerns about bleeding. However, various risk assessment scores are now available and randomized placebo-controlled trials have established the efficacy of low-molecular-weight heparin or the direct oral Xa inhibitors rivaroxaban and apixaban in ambulatory patients with cancer at high risk of venous thromboembolism (VTE). This review provides an overview of (1) primary thromboprophylaxis in the setting of hospitalized surgical and medical patients, (2) extended thromboprophylaxis after hospital discharge, (3) performance of risk assessment tools for CAT, and (4) primary thromboprophylaxis in ambulatory patients with cancer. The aim is to provide support to physicians in identifying ambulatory patients with cancer at high VTE risk who benefit most from medical thromboprophylaxis according to current recommendations from international guidelines.

Wolfgang mondorf, frankfurt: DHR -2025, WAS GIBT ES NEUES?: Die DHR-Webseite (www.pei.de/DE/regulation/melden/dhr/dhr-node.html) beinhaltet neben fünf Publikationen, die letzte von 2020, einen Jahresbericht 2022/2023. Die Daten aus 2023 sind als vorläufig gekennzeichnet. Veröffentlicht sind die Anzahlen gemeldeter Fälle (Hämophilie A/B nach Schweregrad, von Willebrand Syndrom Typ 3 und andere, seltene Faktoren und Hemmkörper bei Kindern und Erwachsenen), sowie der Verbrauch bis 2022. Klinisch relevante Daten, wie z.B. Blutungen, die Teil der Einzelmeldung sind, finden sich nicht.