Should thromboprophylaxis be used, and if so, when should it be introduced? Consequently, DDAVP and tranexamic acid (TXA) have previously been used to manage severe episodes of epistaxis. https://doi.org/10.1182/hematology.2019000065, Orthopedic (eg, joint replacement or amputation), SD, dry heat (80C, 72 h), nanofiltration (35 nM), VWF:RCo and FVIII:C > 50 for 7-10 d; avoid VWF:RCo > 200 and FVIII:C > 250, VWF:RCo and FVIII:C > 30; preferable >50 for 3-5 d, FVIII:C > 50; duration not specified; postoperative period, VWF:RCo and FVIII:C = 80-100 for 36 h; then, >50 for 5-10 d; avoid FVIII:C > 150, Day of surgery: VWF:RCo > 50; FVIII:C > 80-100; days 1-7: VWF:RCo > 30; FVIII:C > 50; days 8-14: VWF:RCo and FVIII:C > 30; avoid FVIII:C > 250, Trough VWF:RCo > 30 and FVIII:C > 50 for 3-5 d. We included 7 case series for major surgeries and 2 RCTs and 12 case series for minor surgeries. Recorded baseline results from the original time of diagnosis at 32 years of age were von Willebrand factor (VWF):antigen (Ag) 26 IU/dL, ristocetin cofactor assay (VWF:RCo) 12 IU/dL, collagen binding (VWF:CB) 24 IU/dL, and factor VIII (FVIII) one stage clotting assay (FVIII:C) 30 IU/dL. Although limited data have been reported to date, these findings suggest that it is useful to objectively assess bleeding phenotype for any patient with VWD undergoing an elective procedure. This plan should clearly outline the planned treatment approach, blood sampling requirements, and monitoring intervals. Future studies should account for number of infusions required to achieve hemostatic FVIII and VWF levels and provide sufficient timepoints for detailed analysis of efficacy. Thereafter, daily VWF:Ag, VWF:RCo, and FVIII:C levels are performed for all patients with VWD after major surgery. Management can vary widely depending on the type of VWD, severity and location of bleeding, and need for invasive procedures. There remains a lack of consensus between best practice guidelines as to the therapeutic target and assays to be monitored in the postoperative period. We included all studies identified as potentially relevant and performed duplicate screening of the full texts for each of the SRs separately. DDAVP is a synthetic analog of vasopressin that stimulates release of VWF from the Weibel Palade bodies of endothelial cells into the circulation. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Learn about diagnosis and specialist referrals for Von Willebrand disease. Knowledge regarding the management of orthopaedic surgery in VWD is limite government site. We included studies in which researchers enrolled patients with all types of VWD or hemophilia and studies that addressed any of the clinical outcomes of interest listed below. An additional dose of 25 IU/kg of VWF concentrate was administered at 12 hours postoperation, and thereafter, trough plasma VWF:RCo levels >50 IU/dL were maintained with once daily blood sampling and dosing for 10 days. Patients with VWD retain the ability to synthesize and secrete endogenous FVIII, which will stabilize after administration of exogenous VWF. The advent of new VWF formulations containing VWF alone without concomitant FVIII will make this knowledge even more important going forward with VWD treatment. This is achieved, because the infused rVWF can bind and stabilize endogenously secreted FVIII. These are case series, and there are no comparisons with other groups. of events and total No. Click here to read the guidelines in full, or scroll to the bottom of this page for more information. 2019 by The American Society of Hematology. Given the lack of high-quality evidence such as RCTs, future research should investigate the importance of maintaining FVIII and/or VWF levels after surgery stratified by type of procedure (eg, dental, mucosal, orthopedic), VWD type or subtype, history of bleeding, and baseline VWF levels. Antifibrinolytic drugs bind to the lysine sites on fibrinogen and promote clot stabilization, helping to reduce bleeding risk for patients with VWD undergoing procedures, particularly those involving mucosal surfaces.10 Clinical reluctance to use TXA is often encountered because of longstanding concerns regarding the risk of thromboembolism. Proportion of procedures in which hemostasis was judged as appropriate, 98% (95% CI, 91%-99%). All target plasma levels are expressed as international units per deciliter. During invasive surgic. The resultant bleeding diathesis is characterized by mucocutaneous bleeding, with easy bruising menorrhagia and epistaxis frequent manifestations of the condition. Most people with the disease are born with it, having inherited it from one or both parents. Nonetheless, clinical studies of the use of pdVWF in surgical settings have demonstrated their hemostatic efficacy.10 Critically, most pdVWF contain variable amounts of FVIII (Table 2). Mannucci PM, Kempton C, Millar C, et al. They may also require this surgery independently of their haemostasis disorder. Castaman G, Goodeve A, Eikenboom J; European Group on von Willebrand Disease. The certainty of the evidence was very low because of the risk of bias, indirectness, and imprecision (very few patients). Subtypes of this disease are characterized by quantitative and/or qualitative abnormalities of von Willebrand factor, a plasma protein essential to platelet adhesion and the stabilization of factor VIII . The .gov means its official. Treatment options for patients with VWD who are undergoing surgery include administration of VWF-containing concentrates (both plasma derived and recombinant), desmopressin to induce release of stored endogenous VWF from the vascular endothelium, and adjunctive antifibrinolytic therapy such as tranexamic acid (TXA).4, In 2017, the American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation (NHF), and the World Federation of Hemophilia (WFH) convened a working group to define the scope and priority areas of focus for updated guidelines on VWD. Von Willebrand disease is a distinct disorder, it is not hemophilia. Recommendations on the perioperative therapeutic targets for patients with VWD undergoing surgery. What are the Symptoms of VWD? For this patient, the Condensed MCMDM-1VWD and International Society on Thrombosis and Hemostasis BAT scores are concordant at 8 (normal reference for males <4),4,5 with a mucocutaneous BS of 5. Platelet von Willebrand factorstructure, function and biological importance. Hemostatic levels are maintained until bleeding risk abates: usually 3 to 5 days for minor procedures and 7 to 14 days for major surgery. Makris M, Colvin B, Gupta V, Shields ML, Smith MP. sharing sensitive information, make sure youre on a federal For dichotomous outcomes, we used the risk ratio and its 95% confidence interval (CI) as the effect measure in comparative studies, and proportions and their 95% CIs in single-arm studies. Baseline bleeding scores (BSs) may assist in identification of patients with a higher risk of postsurgical bleeding. In patients with low VWF levels (plasma VWF levels 30-50 IU/dL), DDAVP induces a predictable and sustained response.7 For patients with type 1 or 2 VWD, responses to DDAVP are higher.16 Failure to respond is more commonly seen in patients with baseline plasma VWF levels <10 IU/dL.17 For patients with increased plasma VWF clearance owing to specific VWF mutations (type 1C VWD) or enhanced susceptibility to cleavage by ADAMTS13 (some patients with type 2A VWD), an initial adequate response to DDAVP may be short lived, underscoring the importance of a trial to assess DDAVP response in advance of surgery.16 The use of DDAVP in type 2B VWD is relatively contraindicated because of concerns of worsening thrombocytopenia.17 For surgery, DDAVP may be administered via the IV or subcutaneous route (off label in the United States), with similar responses seen.18 Because of the risk of dilutional hyponatremia, sodium levels should be checked before repeated use, and fluids should be restricted post-DDAVP (1-1.5 L in 24 hours after use). Many doctors consider DDAVP the first treatment for managing von Willebrand disease. Limitations exist with the use of BATs, with the possibility of underestimation of bleeding phenotype in younger patients or those who have undergone minimal previous hemostatic challenges.1 In contrast, the use of prophylactic treatment to cover previous procedures may result in a falsely elevated BS. Finally, BATs are easily saturable so that the same score is derived for a severe bleeding episode (eg, epistaxis requiring cautery) irrespective of whether it occurred on 1 or multiple occasions.1 For our patient, this represents his first major surgical challenge. Consequently, plasma VWF:Ag levels may correct into the normal range for some patients with quantitative VWD.7,8 Aging-associated increases in plasma VWF:Ag levels have also been observed in some patients with type 2 VWD. Pairs of independent reviewers screened the titles and abstracts of all citations for both SRs. P = .02. 10. Whenever possible, major surgery in patients with VWD should be performed in a center with a hematologist with expertise in the management of bleeding disorders and continuous access to laboratory testing (VWF:Ag, VWF:RCo, and FVIII:C levels).16 A formal written hemostatic management plan should be developed and then communicated to both the patient and the operative team (surgical, anesthetics, and nursing). In general terms, for minor surgery, treatment is usually continued for 1 to 5 days, with 7 to 14 days of hemostatic treatment for major surgery (Table 3).10,16,24,28 In our practice, plasma VWF:Ag, VWF:RCo, and FVIII:C levels are determined by the administration of VWF concentrate preoperatively to ensure adequacy of levels before surgery. However, he has a significant bleeding history that includes recurrent epistaxis (requiring multiple cauterizations), bleeding after dental extraction (requiring packing and suturing), and lifelong easy bruising. A 70-year-old man with type 2M von Willebrand disease (VWD) has failed to attend for a number of years and now requires a right total knee replacement (TKR). What is the role of antifibrinolytic therapy in the perioperative setting? We searched Medline and EMBASE from inception through October 2019 for randomized clinical trials (RCTs), comparative observational studies, and case series that compared maintaining factor VIII (FVIII) levels or von Willebrand factor (VWF) levels at >0.50 IU/mL for at least 3 days in patients undergoing major surgery, and those with options for perioperative management of patients undergoing minor surgery. We assessed indirectness by focusing on characteristics of the population, particularly the proportion of participants who had VWD instead of other bleeding disorders. He has undergone a previous 1-deamino-8-D-arginine vasopressin (DDAVP) trial that demonstrated a partial response (1 hour post-DDAVP: VWF:Ag 78 IU/dL, VWF:RCo 46 IU/dL; 4 hours post-DDAVP: VWF:Ag 52 IU/dL, VWF:RCo 32 IU/dL). VWD is a heterogenous disorder encompassing both quantitative (type 1 and type 3 VWD) and qualitative deficiencies in VWF (type 2 VWD). In the case of our patient, he was treated with 45 IU/kg of pdVWF concentrate preoperatively with preoperative plasma VWF levels of VWF:Ag 128 IU/dL, VWF:RCo 102 IU/dL, and FVIII:C 125 IU/dL. Frequent monitoring of plasma levels and access to hemostatic testing are, therefore, essential for patients undergoing major surgery, particularly with more severe forms of VWD. Perioperative hemorrhage was reported in pediatric studies of orthopedic procedures, but not in adult cohorts undergoing neurosurgical interventions. Three studies reported this outcome; no thrombotic events occurred in any of the 3 studies. Federici AB, Bucciarelli P, Castaman G, et al.. As a library, NLM provides access to scientific literature. In addition, we reported results from case series, but we rated the evidence as very low quality because of the lack of comparative data. Several definitions for bleeding; No. Desmopressin (DDAVP) is the treatment of choice for type 1 vWD as it increases endogenous release of FVIII and von Willebrand factor (vWF) and is . Dosing of VWF replacement is based on body weight and in vivo recovery (IR) of VWF:RCo and FVIII, with 1 IU/kg VWF:RCo or FVIII raising plasma VWF and FVIII levels 2 IU/dL.25 Despite the increased prevalence of obesity, the impact of elevated body mass index on IR in VWD remains uncertain, and dosing is based on actual body weight. 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