Junction: Hematology

Individualized Prophylaxis May Benefit Boys With Severe Hemophilia A

About 40% of boys with hemophilia developed joint changes detectable by magnetic resonance imaging (MRI), especially in the ankles and elbows, despite dose- and frequency-adjusted prophylaxis. This is according to the serial and end-of-study findings from the Canadian Hemophilia Primary Prophylaxis Study, which were reported in Research and Practice in Thrombosis and Haemostasis. The study…

Tranexamic Acid With Emicizumab Prophylaxis May Benefit Hemophilia Patients

Blood loss was lower after tail clip in a mouse model of hemophilia following prophylaxis with emicizumab plus tranexamic acid, according to a new study that was published in the journal Haemophilia.  Compared to treatment only with emicizumab, the addition of tranexamic acid to the prophylaxis significantly reduced joint bleeding. The fibrin fiber diameters of…

Case Report: A Rare Co-occurrence of ALGS and Von Willebrand Disease

A 17-year-old Indian female presented with co-occurring Alagille syndrome (ALGS) and von Willebrand disease (vWD). The likelihood of having both genetic conditions is extremely low, according to a case report published in the South African Journal of Child Health. The patient was diagnosed with ALGS during infancy. The diagnosis was based on phenotypic criteria (ie,…

Surgical Treatments Provide Pain Relief in Hemophilic Arthropathy Patients

Findings from a study comparing the outcomes following total ankle replacement (TAR) and ankle fusion for patients with hemophilia showed significantly lower pain scores in both groups, according to a new study published in Haemophilia. Ten out of the 11 patients who received ankle fusion successfully demonstrated bony consolidation within 3 months. Subjective outcomes using…

Survey: Patients With Hemophilia A Prefer Emicizumab to FVIII Prophylaxis

Researchers have discovered through a survey that patients with hemophilia A overwhelmingly preferred emicizumab treatment to factor VIII (FVIII) prophylaxis, according to a letter to the editor published in Haemophilia. The most common medical approach to treating patients with hemophilia A is through prophylactic infusions of FVIII a few times a week. However, some patients…

Novel Therapy Prevents Inhibitor in Hemophilia A Patients Receiving Recombinant FVIII Treatment

A tolerogenic form of factor VIII (FVIII) is useful as an immunotherapy strategy to prevent inhibitor development in patients with hemophilia A, according to a study published in the Journal of Thrombosis and Haemostasis.  Recombinant FVIII is the first-line treatment for patients with hemophilia A. However, the development of inhibitors is a serious complication because…

Case Report: An Unusual Clinical Presentation of Acquired Hemophilia A

Sokołowska et al reported the rare case of a 57-year-old man diagnosed with acquired hemophilia (AH) whose first clinical manifestations were epileptic seizure and hemorrhage into the ventricular system of the brain. This unusual case was recently published in the Annals of Agricultural and Environmental Medicine. The patient had a history of cerebral infarction, alcoholism,…

hemophilia etiology

Many Hemophilia Carriers Have Abnormal Bleeding Scores

Although the majority of hemophilia carriers have a normal bleeding score, a substantial proportion does not, according to a new study published in Haemophilia. “We believe this analysis contributes significantly to our understanding of bleeding symptoms in hemophilia carriers,” the authors of the study wrote, adding that it also identified the gaps in knowledge that…

Next post in HEM News Briefs