Blood tells a hidden story: Von willebrand disease in the puzzle of refractory postpartum hemorrhage

Abstract

Abstract Blood Tells a Hidden Story: Revealed Von Willebrand Disease in the Puzzle of Refractory Postpartum Hemorrhage Nasim Salimiaghdam1, Ahmed Mustafa1, Mojtaba Akhtari2, David Shcaebler3Internal Medicine Resident Physician, Capital Health Regional Medical Center, Trenton, New JerseyMedical Hemato-Oncology Faculty, Loma Linda University, San Bernardino, CaliforniaHemato-Oncology Faculty, Capital Health Regional Medical Center, Trenton, New Jersey Background: Although immediate postpartum hemorrhage (PPH) is a well-recognized issue, less attention has been given to problems related to isolation of delayed and recurrent PPH initiated greater than 24 hours postpartum, which merits imaging and evaluation for coagulopathy, including evaluation of underlying coagulopathy. Von Willebrand disease (vWD), the most common inherited bleeding disorder, is often undiagnosed in women of reproductive age who can present in an often-indolent manner that dramatically reveals problems in the context of the initial hemostatic stress of childbirth. Case Presentation: We present a 31-year gravida 1 para 1 female who experienced severe delayed PPH initiated 13 days after her uncomplicated cesarean delivery. Over the subsequent four weeks, she was rehospitalized four additional times due to life-threatening hemorrhaging that necessitated several massive transfusion protocols, antifibrinolytics, iron repletion, and replacement of factor VIII with a VWF product (Humate-P). She underwent extensive intervention and imaging, including embolization of a left uterine artery pseudoaneurysm and subsequent prophylactic bilateral embolization of the uterine arteries. Ultimately, she received an emergent hysterectomy after repeated hemorrhaging. Of note, her past medical history and personal bleeding history are significant for epistaxis, easy bruising, and menorrhagia, which is strongly suggestive of an underlying bleeding disorder. Unfortunately, the multiple vWD tests collected in the immediate postpartum period, likely affected by the stress of the medical emergency that raised vWF and inhibited transfused factor levels of VWF, were all normal. Uterine pathology to exclude retained products of conception or malignancy was performed. Conclusion: This case highlights the diagnostic pitfalls of vWD in the postpartum period, where the biochemical derangements can be obscured physiologically and iatrogenically. Clinical history remains the most important factor. If a patient has unexplained delayed PPH and evidence of bleeding diathesis, we would recommend repeating the baseline tests for vWD. Failure to investigate these disorders can lead to a prolonged diagnosis, needless morbidity, and obstetric complications in the future.