Discrepant Hemophilia A: An Underdiagnosed Disease Entity.
The objective was to review the characteristics and the current understanding of mechanisms contributing to assay discrepancy in DHA. METHODS: Characteristics of the DHA patients treated were examined by retrospective chart review. In addition, a literature review was performed to determine the current understanding of DHA. RESULTS: Three cases of DHA were diagnosed based on bleeding phenotype: 2 cases represented missed diagnoses of HA, and 1 represented misclassification of hemophilia severity. The revised diagnosis and classification of hemophilia directly affected clinical management. Review of the literature identified 18 articles with an estimated pooled prevalence of 36% (95% CI, 23%-56%; I2 = 85%; P
This article describes a patient successfully treated with recombinant factor VIIa, porcine factor VIII, plasmapheresis, rituximab, and high-dose corticosteroids.
Patients with hemophilia are at a high risk of developing arthropathy and so are more likely to require surgery during their lifetime; in particular, major orthopedic surgery . The management of patients with hemophilia undergoing surgery has additional considerations, as they are more likely to experience excessive or prolonged bleeding and associated complications, compared with patients without hemophilia .
Classification of the X-linked recessive bleeding disorder Hemophilia A (HA) is based on residual Factor VIII activity level (FVIII:C) that largely correlates with bleeding severity. Understanding the underlying basis for bleeding phenotypes differing from those predicted by FVIII:C should refine classification of patients, clarify genotype-phenotype correlations, and improve clinical management of persons with HA.
Hemophilia A is a bleeding disorder due to the deficiency of coagulation factor VIII (FVIII). According to resident FVIII activity, the disease was divided into mild type (FVIII:c > 5 IU/dL), moderate type (FVIII:c 1–5 IU/dL) and severe type (FVIII:c
Iliacus hematoma syndrome (IHS) is characterized as a retroperitoneal compartment neuropathy caused by bleeding within the iliacus muscle leading to hematoma formation and compression upon the femoral nerve . Subsequently, it shows symptoms such as weakness of the iliopsoas muscle, numbness, loss of knee-jerk, and sensory loss of anteromedial thigh . IHS may be precipitated by trauma alone but have characteristically been described in patients with hemophilia and those on anticoagulation therapy [3, 4].
Condition: Hemophilia A Intervention: Biological: Moroctocog-alfa (AF-CC) Sponsor: Pfizer Recruiting
AbstractBackgroundClotting factor replacement forms the pillar of treatment for children with hemophilia. Most children can be treated using peripheral venipuncture, but very young children and children with poor venous access might require a central venous catheter. Short-term and long-term complications of implantable venous access device placement (also known as port placement) can result in important morbidity and mortality in children with hemophilia.ObjectiveThe purpose of this study is to describe our experience with port placement in children and adolescents with severe hemophilia (
CONCLUSION: Emicizumab enhances coagulation potential in PwMHA. Assessment of ex vivo coagulant activity of emicizumab could be helpful for predicting coagulant potentials prior to treatment in these patients. PMID: 32384547 [PubMed - as supplied by publisher]
ngrande P Abstract Acquired hemophilia A (AHA), a rare bleeding disorder caused by neutralizing autoantibodies against coagulation factor VIII (FVIII), occurs in both men and women without a previous history of bleeding. Patients typically present with an isolated prolonged activated partial thromboplastin time (APTT) due to FVIII deficiency. Neutralizing antibodies (inhibitors) are detected using the Nijmegen-modified Bethesda assay. Approximately 10% of patients do not present with bleeding and, therefore, a prolonged APTT should never be ignored prior to invasive procedures. Control of acute bleeding and preven...