Rapid recognition and optimal management of hemophilia in the emergency department: A quality improvement project.

Objectives: This study aimed to assess the effectiveness of a continuous quality improvement initiative at the University of Florida Health Physicians practice in reducing the time to administer factor replacement therapy (FRT) for hemophilia patients presenting with bleeding in the emergency department (ED).

Methods: The study, a quasi-experimental, interventional design, was conducted between January 2020 and January 2023. The intervention, implemented in September 2021, involved training ED physicians, creating a specialized medication order set within the electronic health record (EHR), and a rapid triage system.

Long-term follow-up of patients with congenital thrombotic thrombocytopenia purpura receiving a plasma-derived factor VIII (Koate) that contains ADAMTS13.

Background: Hereditary thrombotic thrombocytopenia purpura (hTTP) is an ultra-rare disorder resulting from an inherited deficiency of ADAMTS13, a von Willebrand factor (VWF)-cleaving metalloprotease. The plasma-derived factor VIII/VWF Koate (FVIII/VWF ) has been shown to contain ADAMTS13, allowing for its use to treat hTTP at home by the patient/caregiver.

Aim: Based on prior demonstration of safe and effective use of FVIII/VWF in eight patients with hTTP, we conducted a retrospective study to gather additional data regarding the use of FVIII/VWF for hTTP.

Reducing unnecessary premedication prior to parenteral iron therapy: A quality improvement project.

Background: Intravenous (IV) iron carries risks of mild, self-limiting, tryptase-negative Fishbane and complement activation-related pseudo-allergy reactions, with rare reports of anaphylaxis. Historically, high-molecular-weight iron dextran (HMWID) was associated with a higher incidence of anaphylaxis and empiric premedication with antihistamines/corticosteroids have been used to mitigate this risk. HMWID is no longer available and the risk of hypersensitivity reactions with newer IV iron formulations is low.

Real-world safety and effectiveness of recombinant porcine sequence factor VIII in acquired haemophilia A: A non-interventional, post-authorization safety study.

Introduction: Recombinant porcine factor VIII (rpFVIII, susoctocog alfa) is indicated for the treatment of bleeding episodes in adults with acquired haemophilia A (AHA).

Aim: To provide long-term real-world safety and effectiveness data for rpFVIII in the management of AHA bleeding episodes.

Methods: US PASS (NCT02610127) was a multicentre, uncontrolled, open-label, post-marketing safety surveillance study conducted in adults with AHA.

Dysfibrinogenemia: discrepant results following infusion of purified fibrinogen.

Inherited dysfibrinogenemias are molecular disorders of fibrinogen that affect fibrin polymerization. The majority of cases are asymptomatic, but a significant proportion suffer from increased bleeding or thrombosis. We present two unrelated cases of dysfibrinogenemia, both of whom showed a characteristic discrepancy between fibrinogen activity and the immunologic fibrinogen.

Use of cell salvage and HBOC-201 in a pregnant Jehovah's Witness with sickle beta+thalassaemia undergoing emergency caesarean section.

Patients with severe anaemia who refuse or cannot safely receive red cell transfusion present challenges during pregnancy, delivery and the postpartum period. Strategies including HBOC-201 (Hemopure) and intraoperative use of cell salvage have been used in non-pregnant patients to improve oxygen carrying capacity; however, these products pose unique risks in pregnant patients, those with sickle cell disease (SCD) and those undergoing caesarean section (C-section). We describe a case of a pregnant sickle beta+thalasasaemia patient who presented at 27 weeks gestation with pre-eclampsia and severe anaemia.

Efficacy and safety of pre-operative insertion of inferior vena cava filter in patients undergoing bariatric surgery: a systematic review.

Prophylactic placement of inferior vena cava (IVC) filters prior to performing bariatric surgery is an intervention of unclear safety and efficacy with disagreement between current practice guidelines. To better characterize the risk and benefit of IVC filter insertion prior to bariatric surgery based on the current evidence. A systematic review of the literature of patients with prophylactic IVC filter insertion prior to bariatric surgery was performed and 32 studies were identified for inclusion into the review, of which none were randomized controlled trials.

Burnout in US hematologists and oncologists: impact of compensation models and advanced practice provider support.

Burnout is prevalent throughout medicine. Few large-scale studies have examined the impact of physician compensation or clinical support staff on burnout among hematologists and oncologists. In 2019, the American Society of Hematology conducted a practice survey of hematologists and oncologists in the AMA (American Medical Association) Masterfile; burnout was measured using a validated, single-item burnout instrument from the Physician Work-Life Study, while satisfaction was assessed in several domains using a 5-point Likert scale.

A modeling approach to derive baseline risk estimates for GRADE recommendations: Concepts, development, and results of its application to the American Society of Hematology 2019 guidelines on prevention of venous thromboembolism in surgical hospitalized patients.

Objective: The goal of this study was to develop an approach that can be used where baseline risk estimates that are directly applicable to prioritized patient-important outcomes are not available from published studies.

Study Design: The McMaster University GRADE Centre and the ASH guideline panel for the prevention of VTE in surgical patients developed a modeling approach based on explicit assumptions about the distribution of symptoms, anatomical location, and severity of VTE events.

Results: We applied the approach to derive modeled estimates of baseline risk.

The Society for Obstetric Anesthesia and Perinatology Interdisciplinary Consensus Statement on Neuraxial Procedures in Obstetric Patients With Thrombocytopenia.

Because up to 12% of obstetric patients meet criteria for the diagnosis of thrombocytopenia in pregnancy, it is not infrequent that the anesthesiologist must decide whether to proceed with a neuraxial procedure in an affected patient. Given the potential morbidity associated with general anesthesia for cesarean delivery, thoughtful consideration of which patients with thrombocytopenia are likely to have an increased risk of spinal epidural hematoma with neuraxial procedures, and when these risks outweigh the relative benefits is important to consider and to inform shared decision making with patients. Because there are substantial risks associated with withholding a neuraxial analgesic/anesthetic procedure in obstetric patients, every effort should be made to perform a bleeding history assessment and determine the thrombocytopenia etiology before admission for delivery.

Inferior vena cava filters: a framework for evidence-based use.

Venous thromboembolism (VTE) is a common cause of morbidity and mortality. Although most patients can be managed safely with anticoagulation, inferior vena cava filters (IVCFs) represent an important alternative to anticoagulation in a small subset of patients. IVCF use has expanded exponentially with the advent of retrievable filters.

Systems-based hematology: highlighting successes and next steps.

Systems-based hematology is dedicated to improving care delivery for patients with blood disorders. First defined by the American Society of Hematology in 2015, the idea of a systems-based hematologist arose from evolving pressures in the health care system and increasing recognition of opportunities to optimize the quality and cost effectiveness of hematologic care. In this review, we begin with a proposed framework to formalize the discussion of the range of initiatives within systems-based hematology.

Society of Interventional Radiology Clinical Practice Guideline for Inferior Vena Cava Filters in the Treatment of Patients with Venous Thromboembolic Disease: Developed in collaboration with the American College of Cardiology, American College of Chest Physicians, American College of Surgeons Committee on Trauma, American Heart Association, Society for Vascular Surgery, and Society for Vascular Medicine.

Purpose: To provide evidence-based recommendations on the use of inferior vena cava (IVC) filters in the treatment of patients with or at substantial risk of venous thromboembolic disease.

Materials And Methods: A multidisciplinary expert panel developed key questions to address in the guideline, and a systematic review of the literature was conducted. Evidence was graded based on a standard methodology, which was used to inform the development of recommendations.

Pharmacologic thromboprophylaxis in adult patients undergoing neurosurgical interventions for preventing venous thromboembolism.

The impact of pharmacologic prophylaxis for venous thromboembolism in patients undergoing neurosurgical intervention remains uncertain. We reviewed the efficacy and safety of pharmacologic compared with nonpharmacologic thromboprophylaxis in neurosurgical patients. Three databases were searched through April 2018, including those for randomized controlled trials (RCTs) and for nonrandomized controlled studies (NRSs).

Methodology for the American Society of Hematology VTE guidelines: current best practice, innovations, and experiences.

Background: Methods for the development of clinical guidelines have advanced dramatically over the past 2 decades to strive for trustworthiness, transparency, user-friendliness, and rigor. The American Society of Hematology (ASH) guidelines on venous thromboembolism (VTE) have followed these advances, together with application of methodological innovations.

Objective: In this article, we describe methods and methodological innovations as a model to inform future guideline enterprises by ASH and others to achieve guideline standards.

American Society of Hematology 2019 guidelines for management of venous thromboembolism: prevention of venous thromboembolism in surgical hospitalized patients.

Background: Venous thromboembolism (VTE) is a common source of perioperative morbidity and mortality.

Objective: These evidence-based guidelines from the American Society of Hematology (ASH) intend to support decision making about preventing VTE in patients undergoing surgery.

Methods: ASH formed a multidisciplinary guideline panel balanced to minimize bias from conflicts of interest.

Associations between hematology/oncology fellows' training and mentorship experiences and hematology-only career plans.

As the adult hematology and oncology fellowship training pathways have merged in the United States and concerns have arisen about the aging of practicing hematologists, the American Society of Hematology and hematology education leaders are looking to improve their understanding of the factors that contribute to fellows' plans to enter hematology-only careers. With the support of the American Society of Hematology, we collected and analyzed data from a survey of hematology/oncology fellows (n = 626) to examine the relationship between training and mentorship experiences and fellows' plans to enter hematology-only careers. Fellows who planned to enter hematology-only careers were significantly more likely to report having clinical training and mentorship experiences in hematology throughout their training relative to fellows with oncology-only or combined hematology/oncology career plans.