The International Society on Thrombosis and Hemostasis (ISTH) created a Bleeding Assessment Tool (BAT) that is useful to identify and assess disease severity in people with hemophilia, both newly diagnosed patients and those who had a prior diagnosis, a study found.
The study, “Application of the ISTH bleeding score in hemophilia,” was published in Transfusion and Apheresis Science.
Hemophilia is a genetic blood disorder that affects the body’s ability to make blood clots to prevent and heal excessive bleeding. Disease severity can be extremely variable depending on the degree of deficiency in blood-clotting factors. Although patients can lead a normal life with proper treatment and self-care, an early and accurate diagnosis is essential.
“Evaluating the presence and severity of bleeding symptoms is a fundamental step in the assessment of patients’ clinical outcomes. The ISTH/SSC joint committee established a bleeding assessment tool (BAT) to standardize the reporting of bleeding symptoms,” the researchers stated, highlighting that this tool gives comparable results even when patients’ clotting factor levels are not known.
“Limitations of BATs exist, however, in particular for pediatric patients because of a lack of exposure to [bleeding risk events], particularly in mild bleeding disorders,” they said.
In this study, researchers aimed to evaluate the utility of ISTH-BAT in diagnosis, determine disease severity in newly and previously diagnosed patients, compare bleeding scores between adult and pediatric groups, and analyze its relationship with blood-clotting factor levels.
Data showed that bleeding scores were significantly higher in patients with hemophilia A or B compared to healthy control subjects. However, they were identical between patients with hemophilia A and B, and between newly and previously diagnosed patients.
Bleeding scores among pediatric patients and those with milder forms of hemophilia were lower in comparison with adult patients and those affected by severe forms of the disease.
“The use of bleeding assessment tool still has limitations within the pediatric population. In this study, as expected, the bleeding scores were higher in adults compared to children, as they would naturally accumulate more bleeding symptoms and experience more hemostatic challenges like trauma and post-surgical bleeds,” the investigators said.
“The ISTH-BAT can be easily used in the clinics by physicians and can help to identify those patients who should be further investigated. Our ongoing study will show whether the score can predict the risk for bleeding and pediatric and/or adult hemophilia patients. Similarly future research is needed to assess the predictive ability of ISTH for bleeding risk in patients with hemophilia,” they concluded.