Studio Italiano sulla malattia di von Willebrand tipo 3

 

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QuestVWD3-Nov30

Table 1. Assigned score for each bleeding symptom.

SCORE

Symptom

-1

0

1

2

3

4

Epistaxis

No or trivial (less than 5)

> 5 or more than 10’

Consultation only

Packing or Cauterization or Antifibrinolytic

Blood transfusion or Replacement therapy or Desmopressin

Cutaneous

No or trivial (<1 cm)

> 1 cm and no trauma

Consultation only

Bleeding from minor wounds

No or trivial

(less than 5)

> 5 or more than 5’

Consultation only

Surgical hemostasis

Blood transfusion or Replacement therapy or Desmopressin

Oral cavity

No

Referred at least one

Consultation only

Surgical hemostasis or Antifibrinolytic

Blood transfusion or Replacement therapy or Desmopressin

GI bleeding

No

Associated with ulcer, portal hypertension., hemorrhoids, angiodysplasia

Spontaneous

Surgical hemostasis, Blood transfusion, Replacement therapy, Desmopressin, Antifibrinolytic

Tooth extraction

No bleeding in at least 2 extraction

None done or no bleeding in 1 extraction

Referred in <25% of all procedures

Referred in >25% of all procedures, no intervention

Resuturing or packing

Blood transfusion or Replacement therapy or Desmopressin

Surgery

No bleeding in at least two surgeries

None done or no bleeding in 1 surgery

Referred in <25% of all surgeries

Referred in >25% of all procedures, no intervention

Surgical hemostasis or Antifibrinolytic

Blood transfusion or Replacement therapy or Desmopressin

Menorrhagia

No

Consultation only

Antifibrinolytics, Pill use

Dilatation & Currettage, Iron therapy

Blood transfusion or Replacement therapy or Desmopressin or Hysterectomy

Post-partum hemorrhage

No bleeding in at least two deliveries

No deliveries or no bleeding in 1 delivery

Consultation only

Dilatation & Currettage, Iron therapy, Antifibrinolytics

Blood transfusion or Replacement therapy or Desmopressin

Hysterectomy

Muscle hematomas

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

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