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Women and girls with bleeding disorders face multiple bleeding challenges throughout their life. The most significant morbidity and mortality are due to heavy menstrual bleeding and postpartum hemorrhage in their reproductive years. The ASH/ISTH/NHF/WFH 2021 guidelines on diagnosing and managing von Willebrand disease (VWD) provide several new updates.Women with VWD have a higher prevalence of heavy menstrual bleeding. The subpopulation of adolescents is particularly vulnerable, as the diagnosis is often delayed with increased comorbidity of iron deficiency anemia and associated symptoms. A detailed review is done on the prevalence of bleeding-related complications, especially heavy menstrual bleeding (HMB) and post-partum hemorrhage (PPH). The management strategies are also reviewed in detail, with a specific focus on the target factor levels and the use of antifibrinolytics.The 2021 ASH/ISTH/NHF/WFH diagnostic and management recommendations are reviewed with a specific focus on hormonal methods of HMB management and antifibrinolytics in this situation. The reviewed topics include neuraxial anesthesia, factor cutoff, and tranexamic acid use in the postpartum period.

Patients with malignancies including malignant gliomas have a relatively high risk for venous thromboembolism (VTE). Recent evidence has linked isocitrate dehydrogenase (IDH) mutation with reduced VTE risk in malignant glioma. This meta-analysis aims to quantify the association of IDH mutation status with risks of VTE in patients with glioma.We searched PubMed, Google Scholar, Medline OVID, Cochrane library, Cumulative Index to Nursing and Allied Health Literature databases to identify relevant studies. The overall odd ratio (OR) was pooled using the random-effects model. We evaluated the statistical heterogeneity using Cochran's Q statistics and IA total of 2600 patients from 8 studies were included in the meta-analysis. Patients with IDH mutant-type gliomas had a significantly lower risk of VTE (OR: 0.21, 95 % confidence interval [CI]: 0.09-0.46, I2 = 34 %) compared to patients with IDH wild-type gliomas. Among high-grade (III and IV) glioma, VTE events in IDH-mutant gliomas occurred with an OR of 0.28 (95 % CI: 0.14-0.53). No statistically significant decrease in the VTE risk was observed in grade II gliomas with IDH mutation compared to IDH wild-type gliomas, as indicated by the OR of 0.60 (95 % CI: 0.17-2.11).IDH mutation is significantly associated with 79 % lower risk of VTE among patients with high-grade glioma compared to IDH wild-type. Our findings suggest the potential utility of IDH mutation status regarding thromboprophylaxis, and the need for further studies to elucidate the mechanism of the association.

Background Thrombotic disease complicates severe SARS-CoV-2 infection and is associated with increased morbidity and mortality Various anticoagulation strategies have been evaluated in hospitalized patients to prevent complications The impact of chronic anticoagulation prior to SARS-CoV-2 infection on the risk for subsequent thrombosis has not been systematically studied Methods This was a retrospective single center study All patients with positive SARS-CoV-2 PCR testing from March 13, 2020 through May 6, 2020 at the University of Rochester Medical Center were identified We included all patients receiving therapeutic anticoagulation for at least one month prior to COVID diagnosis We documented the rate of thrombotic complications, type of anticoagulation, bleeding complications, and mortality Results A total of 107 SARS-CoV2 infected patients were chronically anticoagulated before SARS-CoV-2 testing with a median age of 78 Of those, 42 required hospital admission with 17 requiring intensive care No patients, inpatient or outpatient, were diagnosed with a new symptomatic thrombotic complication Three patients had minor bleeding in the hospital Thirteen (12%) patients died (69% male) Conclusion Our uncontrolled findings suggest that chronic anticoagulation at the time of infection may protect against thrombotic complications and decrease disease severity

The challenge of pregnancy can be significant to the point of being life-threatening in a woman with a bleeding disorder. Additionally there can be a risk to the fetus and the neonate. A hemostatic defect can affect the course of the pregnancy, but the impact is most feared around delivery in the immediate and the extended post partum period, requiring rapid identification and prompt referral to a hematologist for assistance in management. Identifying the type of congenital bleeding disorder and knowing its inheritance pattern is crucial during counseling prior to conception and in preparation for delivery. A comprehensive approach by a specialized and experienced team in a tertiary care center with access to adequate laboratory monitoring and therapies can facilitate the process. The multidisciplinary team should include a hematologist, an obstetrician, a pediatric hematologist, an anesthesiologist, and in select cases a clinical geneticist and a maternal fetal medicine specialist. In this review article, we will detail the diagnostic path and management of pregnancy and delivery in women with some inherited bleeding disorders, in particular those affected by hemophilia A (HA), hemophilia B (HB), and von Willebrand disease (VWD).

Background Von Willebrand Disease (VWD) is a common inherited bleeding disorder. Patients with VWD suffering from severe bleeding may benefit from the use of secondary long-term prophylaxis. Aim Systematically summarize the evidence on the clinical outcomes of secondary long-term prophylaxis in patients with VWD and severe recurrent bleedings. Methods We searched Medline and EMBASE through October 2019 for relevant randomized clinical trials (RCTs) and comparative observational studies (OS) assessing the effects of secondary long-term prophylaxis in patients with VWD. We used Cochrane Risk of Bias (RoB) tool and the RoB for Non-Randomized Studies of interventions (ROBINS-I) tool to assess the quality of the included studies. We conducted random-effects meta-analyses and assessed the certainty of the evidence using the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach. Results We included 12 studies. Evidence from one placebo controlled RCT suggested that VWD prophylaxis as compared to no prophylaxis reduced the rate of bleeding episodes (Rate ratio [RR], .24; 95% confidence interval [CI], .17–.35; low certainty evidence), and of epistaxis (RR, .38; 95%CI, .21–.67; moderate certainty evidence), and may increase serious adverse events RR 2.73 (95%CI .12–59.57; low certainty). Evidence from four before-and-after studies in which researchers reported comparative data suggested that VWD prophylaxis reduced the rate of bleeding (RR .34; 95%CI, .25–.46; very low certainty evidence). Conclusion VWD prophylaxis treatment seems to reduce the risk of spontaneous bleeding, epistaxis, and hospitalizations. More RCTs should be conducted to increase the certainty in these benefits.

Obesity is associated with endothelial dysfunction, haemostatic and fibrinolytic disturbances, however the impact of obesity on von Willebrand factor (VWF) is unclear.The aim of this study was to test our hypothesis that the prevalence of obesity is higher among participants with low VWF (LVWF) compared to type 1 von Willebrand disease (T1VWD).A retrospective review of the ATHNdataset as of March 2018 was performed. Participants were categorized as T1VWD if their VWF ristocetin cofactor activity was 30 IU/dL and LVWF if the values were 30-50 IU/dL, and by the NIH definitions for body mass index (BMI) for adult participants (≥ 18 years of age) or BMI z-score for paediatric participants ( 18 years).The prevalence of obesity was not significantly different between adults with T1VWD (n = 186) and LVWF (n = 362) (32% vs 36%; p = .345). The mean factor VIII (FVIII) increased with increasing BMIs in both groups. In the paediatric cohort (T1VWD, n = 583; LVWF, n = 1702), there was no difference in the prevalence of obesity, but BMI was positively correlated with mean FVIII (p .001). Children 10 years were 27.6% more likely to be diagnosed with T1VWD compared to 10 years.Among participants in the ATHNdataset, the prevalence of obesity was similar among those with LVWF and T1VWD. However, higher BMI levels were associated with elevated FVIII. Further research is needed to evaluate the impact of obesity on bleeding phenotype and treatment practices.

e19079 Background: Primary myelofibrosis (PMF) carries a poorer prognosis compared to other BCR-ABL-negative myeloproliferative diseases (MPD), and there is increased risk of early mortality due to blast transformation, thrombosis, bleeding complications, and progression of disease. Prior studies report greater incidence of second primary malignancy (SPM) among MPD patients, although the true risk in PMF has not been elucidated. We performed a large database study to evaluate the risk of SPM in PMF patients and analyzed the effects of sociodemographic factors on the risk of SPM. Methods: We used the Surveillance, Epidemiology, and End Results (SEER) database to identify all patients with a histologic diagnosis of PMF from 2009 to 2018. SPM was defined as any subsequent malignancy that developed at least 1 year after the diagnosis of PMF. Using multiple primary standardized incidence ratio (SIR) session of the SEER*Stat software (version 8.3.9), we calculated SIR and absolute excess risk (AER) of SPM for the entire cohort of PMF and also stratified based on age, sex, race, marital status, receipt of chemotherapy, follow-up duration, and year of diagnosis. We generated the 95% confidence intervals (CI) and p-values assuming Poisson distribution of the observed incidences of SPM. Results: A total of 5,273 patients with PMF were included in the analysis, of which 342 patients (6.4%) developed SPM. SPM occurred at SIR of 1.97 (95% CI 1.77-2.18, p60 years (SIR 2.34, 95% CI 1.89-2.86 vs SIR 1.86, 95% CI 1.65-2.10, p 0.01) and for PMF diagnosed after 2009 vs ≤2009 (SIR 2.64, 95% CI 2.26-3.07 vs SIR 1.61, 95% CI 1.40-1.85, p

Uremic platelet dysfunction rarely causes significant bleeding in adequately dialyzed patients. When encountered, the management is complicated by a lack of well-supported treatment modalities. Estrogen use in uremic platelet dysfunction has been described, but enthusiasm for the treatment has been dampened by the risk of thrombotic events in vasculopathic dialysis patients. We present a patient on long-term peritoneal dialysis with coronary disease who developed recurrent life-threatening bleeding episodes secondary to uremia, where treatment with transdermal estrogen was used safely and effectively for a 24-month period.