Two cases of Factor XI deficiency: Use of Thrombin Generation Assays (TGA) to detect a non-bleeding phenotype.

Factor XI deficiency is a rare disorder of hemostasis. Previously also known as "hemophilia C", this defect has been regarded as a risk factor for bleeding. However, it has been known for long that bleeding tendency and severity of bleeding are not related to the residual factor XI activity in symptomatic patients. Moreover, a large proportion of patients with even severe factor XI deficiency are clinically unremarkable and do not show any signs of abnormal bleeding. Here, we present two cases of factor XI deficiency with a non-bleeding phenotype. Adequate diagnostic work-up and evaluation of the bleeding risk are reported and discussed with focus on thrombin generation assays (TGA) for the prediction of bleeding in affected patients. This is of high relevance in affected patients, particularly in the context of surgery.

Concomitant hypofibrinogenemia and factor XI deficiency as rare cause of bleeding during urgent dentistry: case report and short review of the literature.

Hypofibrinogenemia and Factor XI deficiency are rare defects of hemostasis, potentially leading to spontaneous bleeding manifestations and increased bleeding risk during surgery, dentistry, and interventions. Due to the different mode of inheritance, the concomitance of both defects is extremely rare and the clinical management of combined hypofibrinogenemia and factor XI deficiency is not standardized. Here, we report a rare case of concomitant genetically determined hypofibrinogenemia and factor XI deficiency as a cause of increased spontaneous bleeding and bleeding complications during dentistry. The diagnostic procedure including screening assays, single clotting factor determinations, genetic analyses, and also use of thrombin generation assays (TGA) are described. Also, we present our considerations regarding the development of an adequate prophylaxis of bleeding with fibrinogen concentrate in this case. The literature regarding the issue is briefly discussed.

[Anticoagulation after heart valve replacement]. / Antikoagulation nach Herzklappenersatz.

In patients after mechanical heart valve replacement anticoagulation is required for the prevention of thrombotic and thromboembolic events. In this setting oral anticoagulation can only be performed with vitamin K antagonists (VKA), while currently all available non-vitamin K dependent oral anticoagulants (NOAC) are contraindicated in patients with mechanical heart valve replacement. This review deals with anticoagulation in patients with mechanical heart valve replacement as well as coagulation inhibition after bioprosthetic or percutaneous valve replacement. In addition, recommendations are given for antithrombotic medication in patients with mechanical heart valve replacement in various clinical scenarios.

[Acquired inhibitory body hemophilia : A life-threatening reason for the tendency of hematoma-genesis.] / Erworbene Hemmkörperhämophilie : Eine lebensbedrohliche Ursache der Hämatomneigung.

Here, we report a rare case of acquired hemophilia which was diagnosed months after development of spontaneous hematoma. Clinical symptoms, diagnosis, and treatment are briefly summarized. Acquired hemophilia is a rare potentially life-threatening acquired bleeding disorders which should be considered in patients with acquired spontaneous hematoma.

Probleme der Digitalisierung sowie Protest gegen das Finanzstabilisierungsgesetz.

Wiederholt hatte der Vorstand des Berufsverbandes der Deutschen Hämostaseologen (BDDH) in Übereinstimmung mit anderen Berufs- und Ärzteverbänden auf die massiven Probleme der Digitalisierung im Gesundheitswesen hingewiesen. Ärzte waren sich weitgehend einig, dass die Digitalisierung in der aktuellen Form aufgrund fehlender Akzeptanz und massiven technischen Problemen nicht umsetzbar sein wird und zum Scheitern verurteilt ist.

[Cardiovascular risk factors, haemostasis and antithrombotic treatment in retinal vessel occlusion]. / Kardiovaskuläre Risikofaktoren, Gerinnung und gerinnungswirksame Therapien bei retinalen Gefäßverschlusserkrankungen.

Venous and arterial retinal vascular occlusions are age-related disorders, generally associated with classical cardiovascular risk factors, rather than an isolated ocular disease. As affected patients often also have an increased general risk for other vascular diseases, such as ischemic stroke, an interdisciplinary clarification of cardiovascular risk factors and systemic comorbidities is essential for all patients. Extended hemostaseological investigations may be recommended in those patients who do not match the typical risk profile. Patients at a young age by the time of manifestation, without conventional risk factors as well as patients with an increased risk of developing antiphospholipid syndrome may require a selective clinical investigation including testing for thrombophilic risk factors. Recent studies have clearly demonstrated an association between coagulation and lipid metabolism disorders and the development of both retinal vein and artery occlusions in specific subgroups of patients. Therapeutic approaches to treat retinal vascular occlusions or reduce the long-term risk of recurrences with anticoagulant or antiplatelet drugs have not gained widespread acceptance. However, intravenous thrombolysis may be a valuable treatment option for central retinal artery occlusions within a short time to treatment therapeutic window. For defined disorders of the coagulation system, the administration of antithrombotic drugs to reduce the general vascular risk can be a reasonable approach. This article provides an overview of cardiovascular risk factors, the general vascular risk and the current state of knowledge on ophthalmologically relevant disorders of coagulation and lipid metabolism in patients with venous and arterial retinal vascular occlusions.

Hypofibrinogenemia and miscarriage: report of a first successful pregnancy under fibrinogen substitution and short review of the literature.

Disorders of fibrinogen have been reported to be associated not only with bleeding and thrombosis but also with miscarriage. Here, we report the case of a woman with genetically determined hypofibrinogenemia and recurrent miscarriages who had a first successful pregnancy under fibrinogen substitution. Current knowledge on fibrinogen disorders and recurrent miscarriages is briefly summarized and discussed.

Differential Regulation of CD45 Expression on Granulocytes, Lymphocytes, and Monocytes in COVID-19.

CD45 is a transmembrane glycoprotein and protein tyrosine phosphatase expressed on the surface of all nucleated hematopoietic cells. While there is increasing evidence demonstrating the involvement of CD45 in immune system regulation, no information on CD45 expression in inflammation and sepsis is currently available. Therefore, we determined the CD45 surface expression on granulocytes, lymphocytes, and monocytes in patients with COVID-19 and healthy volunteers in both absence and presence of lipopolysaccharide (LPS). Following approval by the local ethics committee, whole blood samples were obtained from patients with COVID-19 infection on day 1 of hospital admission and healthy volunteers. Samples were incubated in absence and presence of LPS and CD45 was measured in granulocytes, lymphocytes, and monocytes using flow cytometry. In comparison with healthy individuals, COVID-19 patients showed an increased CD45 expression on the surface of granulocytes (+35%, p < 0.02) and lymphocytes (+39%, p < 0.0001), but a reduced CD45 expression on monocytes (−35%, p < 0.0001). LPS incubation of whole blood from healthy individuals increased the CD45 expression on granulocytes (+430%, p < 0.0001), lymphocytes (+32%, p = 0.0012), and monocytes (+36%, p = 0.0005), respectively. LPS incubation of whole blood samples from COVID-19 patients increased the CD45 expression on granulocytes and monocytes, and decreased the CD45 expression on lymphocytes. In conclusion, CD45 expression on leucocytes is altered: (1) in COVID-19 patients, and (2) in in vitro endotoxemia in a complex cell-specific way, thus representing a new immunoregulatory mechanism.

Risk Factors for Severe Bleeding Complications in Glaucoma Surgery and the Role of Antiplatelet or Anticoagulant Agents.

Purpose: To evaluate the influences and risk factors for severe bleeding complications during glaucoma surgery, and to investigate the role of antiplatelet (AP) and anticoagulant (AC) agents. Methods: This prospective study enrolled patients undergoing trabeculectomy, trabeculotomy (with Trabectome® or Kahook Dual Blade®), viscocanaloplasty and Ahmed or Baerveldt implants. Bleeding severity was graded on an ordinal scale ranging from 0 to 5. Immediately after surgery and one day later, the incidence and severity of bleeding events was documented on a standardized form. A grade ≥3 was defined as severe bleeding. The influence of known systemic disorders, the type of anesthesia, surgical procedure, intraoperative blood pressure, and the use of or change in AP or AC agents on intraoperative bleeding were analyzed. Results: Data from 89 eyes undergoing glaucoma procedures were included (age 71.3y ± 10.5). We observed severe intraoperative bleeding in 8 eyes (9%) and found that concomitant diseases such as the history of a deep vein thrombosis or peripheral arterial occlusive disease, and the type of surgical procedure (trabeculectomy and viscocanaloplasty) were significantly associated with severe bleeding events. By contrast, the use of AP/ AC agents had no significant influence on severe intraoperative bleeding events. Conclusion: According to the results of our study cohort, glaucoma procedures entailing scleral manipulations (trabeculectomy and viscocanaloplasty) and concomitant diseases such as the history of a deep vein thrombosis or peripheral arterial occlusive disease influence the risk of severe intraoperative bleeding events, we detected no increased risk related to concomitant antiplatelet and/ or anticoagulant medication use.

Acquired hemophilia as a rare cause of excessive bleeding during dentistry: report of two cases and short review.

Acquired hemophilia is a rare coagulation disorder that is not diagnosed by routine clinical laboratory tests. Thus, many perioperative or acute emergent bleeding complications remain unclear until the underlying cause is specified. We report two cases of postoperative bleeding in the context of dental surgery in which subsequent acquired hemophilia could be confirmed and present a short review from the literature.