Are Bleeding Scores Predicting Severity and Outcome in Hemophilia and Rare Bleeding Disorders?

Background: Hemophilia is an inherited severe bleeding disorder, Factors VIII and IX deficiencies have X linked inheritance. Most rare bleeding disorders (RBD) have autosomal recessive inheritance. The ISTH-BAT is a valuable research tool that is applicable to clinical practice and scores patients' bleeding symptoms from 0 to 4. ISTH-BAT has the potential to avoid unwanted laboratory testing, predict the risk of bleeding, describe symptom severity and inform treatment.

Aims: To assess the utility of ISTH-BAT in our bleeding disorder population, compare the bleeding score (BS) in adult and pediatric groups and investigate its association with plasma factor levels.Von Wilebrand disease and thrombasthenia was excluded from this study. One hundred cases of epistaxis whose coagulation workups were within normal limits were enrolled in the control group.

Methods: This is an observational analytical study. The ISTH-BAT was used to calculate bleeding scores (BS) in a group of hemophilic patients and healthy controls. Ethics approval and informed consents were secured prior to the study.

Results: A total of 100 patients, (38 hemophilia A; FVIII deficiency,22hemophilia B; FIX deficiency, 10 hemophilia C; factor XI deficiency, 10 factor VII deficiency, 8 factor X deficiency, 6 factor V deficiency, 5 Afibrinogenemia, Factor I deficiency) and 100 controls were analyzed using the ISTH BAT. Mean BAT score in hemophilia A, B and C were 12.8±8.2; 11.6±8.6; and 7.5 ±5.52respectively.While in RBD were and 7.97±5.56. BAT score in 30 controls was 5.35±4.48. Bleeding Scores were significantly higher in hemophilia A and B patients as compared to controls (P < 0.05) but it was not significant when BAT Scores of hemophilia C patients compared with controls (P=0.35). ANOVA test revealed BAT Score were significantly different among the mild, moderate and severe hemophilia A and B in both adult and pediatric patients but there was no difference in Hemophilia C patients. BAT scores were linked to hematomas; minor wound bleeding in hemophilia A, B patients in the pediatric group while more linked with epistaxis bleeding after dental extraction and surgical interventions in the adult group. Mean BAT Scores of rare bleeding disorders was significantly higher than control group (10.97± 5.65; compared with 5.35±4.48; P<0.05). BAT Score of 10 cases of factor VII deficiency and 5 cases of Afibrinogenemia were higher than other case of RBD, and control group (14.35 ± 7.87; and 13.54±5.56 compared with 5.35±4.48; P< 0.001 and P<0.05respectively).

Conclusions: Our data revealed that the ISTH-BAT can help diagnose the bleeding condition in hemophilia and RBD patients and can be considered a predictor for the bleeding risk or severity. This will decrease need of subspecialty coagulation tests; it ultimately improvesdecision making and the clinical management of patients.