Pregnancy loss in individuals with von Willebrand disease and unspecified mucocutaneous bleeding disorders: a multicenter cohort study.

Background: While bleeding around pregnancy is well described in von Willebrand disease (VWD), the risk of pregnancy loss is less certain.

Objectives: We aimed to describe the frequency of pregnancy loss in females with VWD compared with those with a similar mucocutaneous bleeding phenotype and no VWD or compared with nonbleeding disorder controls.

Methods: Female patients were consecutively approached in 8 specialty bleeding disorder clinics between 2014 and 2023.

Impact of anticoagulation therapy on outcomes in patients with cirrhosis and portal vein thrombosis: A large-scale retrospective cohort study.

Introduction: Portal vein thrombosis in cirrhotic patients presents a significant clinical challenge. This study aims to (1) explore the impact of anticoagulation therapy on patient outcomes; (2) comparative outcomes in portal vein thrombosis treated between direct oral anticoagulant and Vitamin K Antagonist (VKA).

Materials And Methods: We leveraged the TriNetX database to analyze a cohort comprising 4224 patients with liver cirrhosis and PVT who were treated with anticoagulation, alongside a comparison group of 15,300 patients with the same conditions but not receiving anticoagulation therapy.

Retrospective chart review of GI bleeding in people with von Willebrand disease.

Introduction: Gastrointestinal (GI) bleeding events (BEs) in von Willebrand disease (VWD) are difficult to diagnose and often recurrent. Limited data from clinical trials has led to lack of consensus on treatment options.

Aim: Describe current treatments and outcomes for GI BEs in people with VWD.

Standardization of definition and management for bleeding disorder of unknown cause: communication from the SSC of the ISTH.

In many patients referred with significant bleeding phenotype, laboratory testing fails to define any hemostatic abnormalities. Clinical practice with respect to diagnosis and management of this patient cohort poses significant clinical challenges. We recommend that bleeding history in these patients should be objectively assessed using the International Society on Thrombosis and Haemostasis (ISTH) bleeding assessment tool.

Advanced practice providers in hematology: actionable findings from national paired APP and physician surveys.

Advanced practice providers (APPs) are critical to the hematology workforce. However, there is limited knowledge about APPs in hematology regarding specialty-specific training, scope of practice, challenges and opportunities in APP-physician interactions, and involvement with the American Society of Hematology (ASH). We conducted APP and physician focus groups to elucidate major themes in these areas and used results to inform development of 2 national surveys, 1 for APPs and 1 for physicians who work with APPs.

Observational cohort study of long-term outcomes of liver transplantation in haemophilia.

Introduction: Gene therapy is now a reality for individuals with haemophilia, yet little is known regarding the quality-of-life impact of factor correction. As few data exist, and recognizing the analogy to liver transplantation (OLTX), we identified OLTX+ and OLTX- men in the ATHNdataset to compare post-OLTX factor VIII and IX on quality of life (QoL) by Haem-A-QoL and PROMIS-29.

Methods: OLTX- were matched to OLTX+ by age, race, and haemophilia type and severity.

Acquired bleeding disorders secondary to immune checkpoint inhibitors: a case report and systematic literature review.

Acquired bleeding disorders because of an autoimmune phenomenon are rare events. Acquired von Willebrand disease (aVWD) has been estimated as having a prevalence of 400 per million in the general population. Acquired hemophilia A (AHA), the most common of the acquired hemophilias, has an estimated incidence of 1.

The diagnosis, natural history, and management of von Willebrand disease in women in the age of guidelines.

Introduction: 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.

Depression and anxiety in persons with Von Willebrand disease.

Objective: We assessed sociodemographic and clinical characteristics associated with depression and anxiety in individuals with Von Willebrand disease (VWD) aged ≥12 years.

Methods: The study collected data on patients' sociodemographic, joint problems and health-related quality of life (HRQoL) using EQ-5D-3L, 8-item patient health questionnaire for depression and 7-item Generalized Anxiety Disorder Questionnaire from participants in seven geographically diverse US haemophilia treatment centres.

Results: Analyses included 77 participants.

Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis in a postpartum patient with preeclampsia: a case report.

Hemophagocytic lymphohystiocytosis is an extremely life-threatening immune deregulatory syndrome. It is characterized by pathologic activation of immune cells, leading to excessive cytokine production, multiorgan failure, and potentially, death. A 28-year-old primigravida at 32 weeks and 3 days of gestation presented with newly-diagnosed preeclampsia with severe features, fever, and fetal tachycardia.

Risk of second primary malignancy in patients with primary myelofibrosis: a SEER database study.

Prior studies report a greater incidence of second primary malignancy (SPM) among patients with myeloproliferative neoplasms, although the true risk in primary myelofibrosis (PMF) has not been elucidated. We utilized the Surveillance, Epidemiology, and End Results database to evaluate the risk of SPM in PMF patients and analyzed the effects of sociodemographic factors on the risk of SPM. Out of 5273 patients, 385 patients (7.

Managing Pregnant Women with Hemophilia and von Willebrand Disease: How Do We Provide Optimum Care and Prevent Complications?

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.

Isocitrate dehydrogenase mutation and risk of venous thromboembolism in glioma: A systematic review and meta-analysis.

Background: 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.

Does a Bleeding Disorder Lessen the Efficacy of the 52-mg Levonorgestrel-Releasing Intrauterine System for Heavy Menstrual Bleeding in Adolescents? A Retrospective Multicenter Study.

Purpose: The aim of this study is to compare the patient-reported bleeding outcomes and complication rates with the use of the 52-mg levonorgestrel-releasing intrauterine system (52-LNG-IUS) for treatment of heavy menstrual bleeding (HMB) among adolescents with and without a diagnosed inherited bleeding disorder (BD) within the first 12 months after insertion.

Methods: Retrospective chart review was conducted of adolescents ages 14-21 years, with and without an inherited BD, who underwent 52-LNG-IUS insertion between September 2013 and February 2020 for the treatment of HMB.

Results: One hundred forty-four 52-LNG-IUS insertions among 139 subjects were evaluated.