Heyde’s syndrome

Gastrointestinal bleeding is a severe and potentially life-threatening condition associated with significant morbidity and mortality. In clinical practice, its differential dia­gnosis can sometimes be complicated, especially when bleeding is recurrent. One of the lesser-known reasons for recurrent gastrointestinal bleeding is the so called Heyde’s syndrome. It is a combination of bleeding from gastrointestinal angiodysplasia, aortic stenosis and acquired type 2A von Willebrand syndrome. In the submitted article, authors present a summary of the current knowledge on etiology and pathogenesis of the disease. The degenerative aortic valve disease, typically present in elderly patients, causes changes in the blood flow through stenotic opening. This results in elongation of otherwise globular von Willebrand’s factor (vWF) molecules, resulting in its reduced efficacy in hemostasis. According to new scientific discoveries, vWF also plays a role in angiogenesis and therefore in gastrointestinal angiodysplasia formation. This combination of factors results in recurrent bleeding typical for Heyde’s syndrome. Surgical aortic valve replacement is the gold standard treatment. A vicious circle can often be created between the gastroenterologist, who refers the patient for cardiac surgery procedures, and the cardiothoracic surgeon, who is waiting for a time when the patient will stop bleeding. A close communication and cooperation between the gastroenterologist, hematologist and cardiothoracic surgeon is required to properly manage the patient. The presence of angiodysplasia in an elderly patient, associated with recurrent gastrointestinal bleeding, should lead clinicians to look for aortic stenosis and consider Heyde’s syndrome as a potential dia­gnosis. Its interesting etiopathogenesis and rare occurrence led the authors to further investigate this topic. They also present a short summary of their own group of patients. Key words: Heyde’s syndrome – aortic stenosis – angiodysplasia – von Willebrand’s syndrome

Cryoprecipitate Usage and Cost at a Tertiary Care Hospital Between 2010 and 2017

Introduction Cryoprecipitate (CRYO), a blood product prepared from FFP, is rich in fibrinogen, von Willebrand’s factor, and factors VIII and XIII. ​The major indication for CRYO transfusion is fibrinogen deficiency with increased risk of bleeding when fibrinogen is <100 mg/dL, a transfusion trigger rarely followed by the house staff or other clinicians. To limit wastage, Transfusion Services issue them as pools of five individual CRYO units. The use of CRYO at Robert Wood Johnson University Hospital (RWJUH) has significantly risen since 2010, so we conducted a study to identify the factors leading to increased CRYO transfusions. Methods Data on CRYO transfusions from 2010 to 2017 were obtained and analyzed for trends in overall usage and by nursing unit/departments. Cost-analysis was performed to determine the cost of CRYO usage. Conclusion Overall, CRYO usage has risen since 2010, with a sharp increase between 2015 and 2017 attributed to increased transfusions in the operating room (OR), surgical intensive care unit, and hematology-oncology units. There was a sixfold rise in transfusions in the hematology-oncology units. During this time, the lower limit of the fibrinogen reference range decreased from 234 to 190 mg/dL along with a drop in the number of trauma alerts. Values less than the lower reference range were used as the transfusion trigger by some overzealous hematology fellows. Cost-analysis showed that RWJUH lost 75% revenue per patient based on Medicare reimbursement despite efforts to limit wastage and lowering purchasing cost per unit by changing to a new blood supplier. The major determinants of intensified CRYO transfusions at RWJUH are (1) changes in the fibrinogen reference range concomitant with enhanced awareness, monitoring, and early treatment of hypofibrinogenemia, particularly in hematology-oncology units and (2) the complexity of surgical procedures (ie, VAD procedures) requiring more CRYO transfusions rather than the number of trauma alerts.

Ocular bleeding in an undiagnosed hemophiliac neonate causing irreversible loss of vision: A case report with review of the literature

Introduction: Suprachoroidal hemorrhage is an unexpected complication after penetrating keratoplasty during childhood. We report the case of delayed suprachoroidal hemorrhage after penetrating keratoplasty in a neonate as the first manifestation of hemophilia. Case report: A 4-day-old neonate was referred to our hospital because of bilateral corneal opacities present since birth. A diagnosis of bilateral Peter’s anomaly type 2 with right eye corneal perforation was made after examination. Appropriate treatment was commenced, and later, right eye therapeutic penetrating keratoplasty combined with lensectomy, and anterior vitrectomy was performed. This was complicated postoperatively with suprachoroidal hemorrhage and retinal detachment. Conservative management was advised due to poor visual prognosis. Three months later, the patient was found to have high intraocular pressure in the left eye and glaucoma surgery was recommended. Proper hematological investigations before glaucoma surgery in the left eye revealed a low level of factor VIII with normal levels of factor IX and von Willebrand’s factor antigen. Therefore, a diagnosis of hemophilia type A was established. Conclusion: This case serves as a reminder that the occurrence of suprachoroidal hemorrhage, especially in the absence of other predisposing risk factors, should warrant detailed systemic assessment to exclude underlying bleeding disorders.