Table 2.
Score | |||||
---|---|---|---|---|---|
Symptoms (Up to Time of Diagnosis) | 0§ | 1§ | 2 | 3 | 4 |
Epistaxis | No/trivial | >5/year or >10 minutes | Consultation only* | Packing/cauterization or antifibrinolytic | Blood transfusion, replacement therapy^^ or desmopressin |
Cutaneous | No/trivial | ≥5 bruises of >1 cm in exposed areas | Consultation only* | Extensive | Spontaneous haematoma requiring blood transfusion |
Bleeding from minor wounds | No/trivial | >5/year or >10 minutes | Consultation only* | Surgical hemostasis | Blood transfusion, replacement therapy^^ or desmopressin |
Oral cavity | No/trivial | Present | Consultation only* | Surgical hemostasis or antifibrinolytic | Blood transfusion, replacement therapy^^ or desmopressin |
Gastro intestinal bleeding | No/trivial | Present (not associated with ulcer, portal hypertension, hemorrhoids, angiodysplasia) | Consultation only* | Surgical hemostasis or antifibrinolytic | Blood transfusion, replacement therapy^^ or desmopressin |
Hematura | No/trivial | Present (macroscopic) | Consultation only* | Surgical hemostasis, iron therapy | Blood transfusion, replacement therapy^^ or desmopressin |
Tooth extraction | No/trivial or none done | ≤25% of all procedures, no intervention** | >25% of all procedures, no intervention** | Resuturing or packing | Blood transfusion, replacement therapy^^ or desmopressin |
Surgery | No/trivial or none done | ≤25% of all procedures, no intervention** | >25% of all procedures, no intervention** | Surgical hemostasis or antifibrinolytic | Blood transfusion, replacement therapy^^ or desmopressin |
Menorrhagia | No/trivial | Consultation only* or Changing pads > every 2 hours or Clot and flooding or PBAC score >100# | Time off work/school >2/year or Antifibrinolytics/hormonal/iron therapy | Combined treatment with antifibrinolytics and hormonal therapy or Present since menarche and >12 months | Acute menorrhagia requiring hospital admission and emergency treatment or Blood transfusion, replacement therapy^^ or desmopressin or Dilatation and curettage or endometrial ablation or hysterectomy |
Post-partum hemorrhage | No/trivial or no deliveries | Consultation only* or Use of syntocin or Lochia >6 weeks | Iron therapy or Antifibrinolytics | Blood transfusion, replacement therapy^^ or desmopressin or Examination under anaesthesia and/or uterine balloon/package to tamponade uterus | Any procedure requiring critical care or surgical intervention (eg hysterectomy, internal iliac artery legation, uterine artery embolization, uterine brace sutures) |
Muscle haematomas | Never | Post trauma, no therapy | Spontaneous, no therapy | Spontaneous or traumatic, requiring desmopressin or replacement therapy^^ | Spontaneous or traumatic, requiring surgical intervention or blood transfusion |
Hemarthrosis | Never | Post trauma, no therapy | Spontaneous, no therapy | Spontaneous or traumatic, requiring desmopressin or replacement therapy^^ | Spontaneous or traumatic, requiring surgical intervention or blood transfusion |
CNS bleeding | Never | Subdural, any intervention | Intracerebral, any intervention | ||
Other bleeding^ | No/trivial | Present | Consultation only* | Surgical hemostasis, antifibrinolytics | Blood transfusion or replacement therapy^^ or desmopressin |
Notes: In addition to the guidance offered by the table, it is mandatory to refer to the text for more detailed instructions. §Distinction between 0 and 1 is of critical importance. Score 1 means that the symptom is judged as present in the patient’s history by the interviewer but does not qualify for a score 2 or more. *Consultation only: the patient sought medical evaluation and was either referred to a specialist or offered detailed laboratory investigation. **Example: 1 extraction/surgery resulting in bleeding (100%): the score to be assigned is 2; 2 extractions/surgeries, 1 resulting in bleeding (50%): the score to be assigned is 2; 3 extractions/surgeries, 1 resulting in bleeding (33%): the score to be assigned is 2; 4 extractions/surgeries, 1 resulting in bleeding (25%): the score to be assigned is 1. #If already available at the time of collection. ^Include: umbilical stump bleeding, cephalohematoma, cheek hematoma caused by sucking during breast/bottle feeding, conjunctival hemorrhage or excessive bleeding following circumcision or venipuncture. Their presence in infancy requires detailed investigation independently from the overall score. ^^Use of hemostatic blood components and rFVIIa. Reproduced from Rodeghiero F, Tosetto A, Abshire T, Arnold DM, Coller B, James P, Neunert C, Lillicrap D; ISTH/SSC joint VWF and Perinatal/Pediatric Hemostasis Subcommittees Working Group. ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. J Thromb Haemost. 2010;8(9):2063–2065. © 2010 International Society on Thrombosis and Haemostasis.13