Bernard-Soulier syndrome is a rare inherited blood clotting disorder characterized by unusually large, giant platelet cells, thrombocytopenia, and prolonged bleeding time. Due to the rarity of the disease, it may not appear foremost on the clinician’s differential. Affected individuals tend to bleed excessively and incur spontaneous ecchymoses. General coagulation studies may not be helpful, and the paucity of data may obscure the diagnosis. Acumen, supplemented by suspicion, may be the only way to approach this disorder. The disease is familial, and the diagnosis of one patient, the proband, necessitates the consideration of family members as well. This activity reviews the evaluation and treatment of Bernard-Soulier syndrome and highlights the role of the interprofessional healthcare team in evaluating and managing patients with this condition. Clinicians participating in this activity will gain an understanding of the defect in the GPIb-IX-V complex, a critical platelet receptor complex with multifaceted roles in von Willebrand factor binding, thrombosis, and hemostasis. By delving into the intricacies of this rare blood clotting disorder, healthcare professionals will enhance their acumen and suspicion skills, crucial for accurate identification and subsequent multidisciplinary treatment.
- Provider:StatPearls, LLC
- Activity Link: https://www.statpearls.com/ArticleLibrary/viewarticle/18225
- Start Date: 2023-09-01 05:00:00
- End Date: 2023-09-01 05:00:00
- Credit Details: AMA PRA Category 1 Credit™️: 1.5 hours
Nursing: 1.5 hours
Pharmacy: 1.5 hours - MOC Credit Details: ABS - 1.5 Point; Credit Type(s): Accredited CME (ABS)
ABPATH - 1.5 Point; Credit Type(s): Lifelong Learning (ABPATH)
ABA - 1.5 Point; Credit Type(s): Lifelong Learning (ABA)
ABIM - 1.5 Point; Credit Type(s): Medical Knowledge (ABIM)
ABS - 1.5 Point; Credit Type(s): Self-Assessment (ABS)
ABP - 1.5 Point; Credit Type(s): Lifelong Learning and Self-Assessment (ABP) - Commercial Support: No
- Activity Type: Enduring Material
- CME Finder Type: Online Learning
- Fee to Participate: Variable
- Measured Outcome: Learner Knowledge, Learner/Team Competence
- Provider Ship: Directly Provided
- Registration: Open to all
- Specialty: Adolescent Medicine, Ambulatory/Outpatient, Critical Care Medicine, Gastroenterology, General Operative Anesthesia, General Pediatrics, General Surgery, Hematology, Hematology (Blood, BM), Hemostasis & Thrombosis/Coagulation, Hospital Medicine, Internal Medicine, Molecular Genetic Pathology, Pediatric Critical Care Medicine, Pediatric Emergency Medicine, Pediatric Hematology-Oncology, Surgical Critical Care