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Von Willebrand Disease
Published in Vincenzo Berghella, Maternal-Fetal Evidence Based Guidelines, 2022
There is an increased risk of both primary (within 24 hours of delivery) and secondary (>24 hours after delivery) postpartum hemorrhage. vWF and factor VIII levels return to baseline 1–3 weeks following delivery, thus close monitoring and additional doses of DDAVP or vWF/factor VIII concentrates may be warranted [15]. Oral antifibrinolytic therapy can also be used to prevent delayed postpartum bleeding [11]. Patients with type 2B VWD also have an increased risk of morbidity related to primary and secondary postpartum hemorrhage as well as severe thrombocytopenia [16]. Products that inhibit platelet adhesion, such as nonsteroidal anti-inflammatory drugs, should be avoided [8]. As the neonate has up to a 50% chance of having VWD, circumcision should be delayed until VWD status is determined [8].
Trauma Physiology and Metabolism
Published in Ian Greaves, Keith Porter, Jeff Garner, Trauma Care Manual, 2021
Ian Greaves, Keith Porter, Jeff Garner
Keeping the patient warm may mitigate the degree of cooling. The body’s fibrin removal systems which prevent too much clot formation can stop working all together leading to thrombosis or can work too well, prematurely removing clots and allowing bleeding to resume. Treatment with the anti-fibrinolytic drug tranexamic acid as soon as possible after injury reduces the likelihood of death from bleeding, though it must be given within three hours to be effective. If the patient survives the initial trauma, coagulation then gradually returns to normal, followed by a period of increased risk of venous thromboembolism due to the inflammatory stimulus of injury and relative immobility during their hospital stay. An increased risk of thrombosis can be managed by the use of low molecular weight heparin as thromboprophylaxis starting sometime after the initial haemorrhage has been controlled.
Tranexamic Acid (TA)
Published in John C. Petrozza, Uterine Fibroids, 2020
John Storment, Camille Storment
Data show the presence of extensive fibrinolysis in the menstrual blood of women with heavy menstrual bleeding [3]. This has prompted investigation into the use of antifibrinolytic agents to decrease menorrhagia by increasing clot formation [4,5]. Activation of the clotting cascade occurs at the site of tissue injury. It involves the formation of thrombin, which cleaves fibrinogen to fibrin and produces hemostasis. Dissolution of this clot is then activated by fibrin in an effort to keep the vessel open. Fibrinolysis occurs when plasminogen (trapped within the clot) binds to lysine, resulting in a degradation of the fibrin clot. Excessive fibrinolysis can be prevented by tranexamic acid (TA) keeping the clot more stable [6]. TA, a synthetic derivative of the amino acid lysine, binds to plasminogen and blocks the interaction of plasmin with fibrin, thereby preventing clot dissolution [6]. Although its mechanism of action raises concern about an increased risk of thrombosis, this association has not been seen in clinical trials [7].
Gastrointestinal bleeding in von Willebrand patients: special diagnostic and management considerations
Published in Expert Review of Hematology, 2023
Edwin Ocran, Nicholas L.J. Chornenki, Mackenzie Bowman, Michelle Sholzberg, Paula James
With respect to the acute treatment of GI bleeding in VWD, management is similar to other severe VWD-related bleeding commonly seen in type 2A and type 3 VWD patients and typically involves the use of desmopressin, VWF concentrates and recently recombinant VWF (rVWF). Desmopressin, a synthetic analog of the hormone vasopressin, causes the release of stored VWF from endothelial cells, and although a very effective treatment in type 1 VWD patients, has limited efficacy in type 2 VWD, with the risk of worsening thrombocytopenia in type 2B patients, and a general unresponsiveness in patients with type 3 VWD [87,88]. Side effects such as tachyphylaxis and fluid overload limit its use [87,88]. VWF replacement therapies have been shown to be safe and highly effective in treating severe bleeding and may be used as prophylaxis in severe VWD [89]. Adjunctive therapies with antifibrinolytic agents such as tranexamic acid and hormonal contraceptives remain useful additions to treatment as reviewed by James AH [90]. These traditional therapies, however, are reported to be less effective in treating angiodysplastic GI bleeding [31,57].
Building the foundation for a community-generated national research blueprint for inherited bleeding disorders: research to advance the health of people with inherited bleeding disorders with the potential to menstruate
Published in Expert Review of Hematology, 2023
Maureen K. Baldwin, Homa K. Ahmadzia, Diane L. Bartlett, Debbie Bensen-Kennedy, Vidhi Desai, Kristina M. Haley, Sherry L. Herman-Hilker, Amanda M. Kilgore, Roshni Kulkarni, Michelle Lavin, Shari Luckey, Kristen A. Matteson, Kristin Paulyson-Nuñez, Claire S. Philipp, Sachiko Ragosta, Kimberly Rosen, Dawn Rotellini, Angela C. Weyand
Pregnancy and post-partum were accorded their own focused research domain in addition to being a focus in lifespan sex biology (Table 2). The dissemination of feasible tools to diagnose hemorrhage in PPM during pregnancy and in the post-partum context is a prerequisite to conducting research into the prevention of post-partum hemorrhage (PPH) and relevant hemostatic mechanisms (Figure 4b). This research may then investigate the optimization of coagulation factor or antifibrinolytic agent prophylaxis in the context of PPH or for first-trimester procedures, how and when coagulation factor levels change throughout pregnancy and the post-partum period, and the roles of primary versus secondary hemostasis in pregnancy and PPH. The risk of venous thromboembolism (VTE) in post-partum PPM with BDs in the context of hormonal and/or antifibrinolytic treatments requires specific investigation. Widely feasible blood loss quantitation methodology is also key to determining the screening criteria for performing a BD workup, and to optimizing the identification of bleeding problems related to pregnancy.
The management of hematologic malignancies during the COVID-19 pandemic
Published in Expert Opinion on Pharmacotherapy, 2021
Iwona Hus, Aleksander Salomon-Perzyński, Krzysztof Tomasiewicz, Tadeusz Robak
The management of anemia in cancer patients might be complicated by the expected shortage of blood products. Therefore, a more restrictive threshold for RBC transfusion, e.g., Hgb <7 g/dL is suggested, though the patients with severe anemia-related symptoms should receive RBC transfusion even at Hgb level > 7 g/dL [44]. Wider use of long-acting ESA is suggested, as this may avoid the need for additional clinic visits. NCCN experts suggest broadening the use of ESA therapy ± iron supplementation in the short term to manage the anemia in cancer patients requiring blood transfusion support [39]. The lowest dose of ESA sufficient to avoid transfusion, and hence reduce the risk of thrombosis, should be used; therefore, ESA should be withheld in patients with Hgb >10 g/d [NCCN]. The threshold should be lowered also for platelet transfusion (< 10 G/L), except for patients with bleeding. Prophylactic antifibrinolytics could be considered in patients with low platelet count when no platelets are available. Antifibrinolytics should be used in patients with platelet counts >30 G/l and in patients with embolic stroke, thromboembolism, or urinary tract infection.