Practical-Haemostasis.com

Introduction

Rare Bleeding Disorders [RBDs] account for 3-5% of all inherited coagulation disorders and are usually transmitted as autosomal recessive traits. In some countries the prevalence is higher due to consanguineous marriage.
The Rare Bleeding Disorders [RBDs] include:
    - Fibrinogen deficiency
    - Factor II [Prothrombin deficiency]
    - Factor V deficiency
    - Factor VII deficiency
    - Factor V and VIII deficiency
    - Factor XI deficiency
    - Factor XIII deficiency
The bleeding manifestations that individuals with a rare bleeding disorder exhibit, vary significantly both between disorders and in patients with the same disorder. Bleeding symptoms including gum bleeding, expistaxes, menorrhagia and bleeding from minor wounds, are also frequently reported in healthy individuals
A Lifelong Clinical Bleeding Scoring System for the Rare Bleeding Disorders [EN-RBD-BSS] was developed both to identify individuals with a rare bleeding disorder but in addition to separate individuals with a bleeding disorder from those without one.

The algorithm was developed from data derived from 492 patients with a Rare Bleeding Disorder [RBD] enrolled in the European Network of Rare Bleeding Disorders [EN-RBD]. For more information on how the algorithm was developed - see References.

Epistaxis
Epistaxis	No Yes	0 points 1 point
Frequency >1/year	No Yes	0 points 1 point
Spontaneous	No Yes	0 points 1 point
On prophylaxis	No Yes	0 points 1 point
Diffuse Bleeding1	No Yes	0 points 1 point
Bilateral	No Yes	0 points 1 point
Requiring treatment with packing, cauterization or antifibrinolytics	No Yes	0 points 1 point
Requiring treatment with blood transfusion or replacement therapy or desmopressin	No Yes	0 points 2 points
Epistaxis Index Score [Epi_In] [Maximum Sum: 9 points]

¹Diffuse bleeding that lasts at least 10 minutes and/or requires medical attention in the case of epistaxis or oral cavity bleeding; a haematoma >3cm that is considered disproportionate to the causative trauma, when it is not possible to establish a localised lesion and define the haemorrhage volume in the case of CNS bleeding.

Oral Cavity Bleeding2
Oral Cavity Bleeding	No Yes	0 points 1 point
Frequency >1/year	No Yes	0 points 1 point
Spontaneous	No Yes	0 points 1 point
On prophylaxis	No Yes	0 points 1 point
Diffuse Bleeding1	No Yes	0 points 1 point
Involving tongue	No Yes	0 points 1 point
Requiring treatment with surgical haemostasis or antifibrinolytics	No Yes	0 points 1 point
Requiring treatment with blood transfusion or replacement therapy or desmopressin	No Yes	0 points 2 points
Oral Cavity Bleeding Index Score [Oral_In] [Maximum Sum: 9 points]

¹Diffuse bleeding that lasts at least 10 minutes and/or requires medical attention in the case of epistaxis or oral cavity bleeding; a haematoma >3cm that is considered disproportionate to the causative trauma, when it is not possible to establish a localised lesion and define the haemorrhage volume in the case of CNS bleeding.

²Oral cavity bleeding includes gum bleeding, tooth eruption, spontaneous tooth loss bleeding, bleeding occurring after bites/wounds to the lips, cheek and tongue.
Gum bleeding due to a toothbrush should be evaluated as normal; gum bleeding should be considered significant when it causes bloody sputum.

Bruising
Bruising	No Yes	0 points 1 point
Frequency >1/year	No Yes	0 points 1 point
Spontaneous	No Yes	0 points 1 point
On prophylaxis	No Yes	0 points 1 point
Size >5cm	No Yes	0 points 1 point
Involving head or thorax	No Yes	0 points 1 point
Requiring consultation	No Yes	0 points 1 point
Bruising Index Score [Bruis_In] [Maximum Sum: 7 points]

Haematoma
Haematoma	No Yes	0 points 1 point
Frequency >1	No Yes	0 points 1 point
Spontaneous	No Yes	0 points 1 point
On prophylaxis	No Yes	0 points 1 point
Diffuse bleeding1	No Yes	0 points 1 point
Requiring treatment with replacement therapy or desmopressin	No Yes	0 points 1 point
Requiring treatment with surgery + blood transfusion or replacement therapy	No Yes	0 points 2 points
Haematoma Index Score [Haemato_In] [Maximum Sum: 8 points]

¹Diffuse bleeding that lasts at least 10 minutes and/or requires medical attention in the case of epistaxis or oral cavity bleeding; a haematoma >3cm that is considered disproportionate to the causative trauma, when it is not possible to establish a localised lesion and define the haemorrhage volume in the case of CNS bleeding.

Hemarthrosis
Hemarthrosis	No Yes	0 points 1 point
Frequency >1	No Yes	0 points 1 point
Spontaneous	No Yes	0 points 1 point
On prophylaxis	No Yes	0 points 1 point
Disabling3	No Yes	0 points 1 point
Requiring treatment with replacement therapy or desmopressin	No Yes	0 points 1 point
Requiring treatment with surgery + blood transfusion or replacement therapy	No Yes	0 points 2 points
Hemarthrosis Index Score [Haemar_In] [Maximum Sum: 8 points]

³Disabling means that hemarthrosis has led to compromised motility.

Gastrointestinal Bleeding
Gastrointestinal Bleeding	No Yes	0 points 1 point
Frequency ≥2 times	No Yes	0 points 1 point
Spontaneous4	No Yes	0 points 1 point
On prophylaxis	No Yes	0 points 1 point
Causing anaemia	No Yes	0 points 1 point
Requiring treatment with antifibrinolytics	No Yes	0 points 1 point
Requiring treatment with surgical hemostasis, blood transfusion, replacement therapy or desmopressin	No Yes	0 points 2 points
Gastrointestinal Bleeding Index Score [GI_In] [Maximum Sum: 8 points]

⁴Any gastrointestinal bleeding that is not explained by the presence of a specific disease hould be considered as spontaneous. Bleeding associated with an ulcer, portal hypertension,hemorrhoids or angiodysplasia should be considered as not spontaneous.
Melena and hematemesis, if not associated with the conditions listed above, should be considered as possible signs of a bleeding disorder.

CNS Bleeding
CNS Bleeding	No Yes	0 points 1 point
Spontaneous	No Yes	0 points 1 point
On prophylaxis	No Yes	0 points 1 point
Diffuse bleeding1	No Yes	0 points 1 point
Intracerebral bleeding	No Yes	0 points 1 point
Requiring treatment with replacement therapy	No Yes	0 points 1 point
Requiring treatment with surgical hemostasis + blood transfusion or replacement therapy	No Yes	0 points 1 point
CNS Bleeding Index Score [CNS_In] [Maximum Sum: 7 points]

¹Diffuse bleeding that lasts at least 10 minutes and/or requires medical attention in the case of epistaxis or oral cavity bleeding; a haematoma >3cm that is considered disproportionate to the causative trauma, when it is not possible to establish a localised lesion and define the haemorrhage volume in the case of CNS bleeding.

Menorrhagia
Menorrhagia	No or Male Yes	0 points 1 point
Frequency ≥ 1/year	No or Male Yes	0 points 1 point
Spontaneous	No or Male Yes	0 points 1 point
On prophylaxis	No or Male Yes	0 points 1 point
Pictorial chart ≥ 185	No or Male Yes	0 points 1 point
Requiring treatment with antifibrinolytics, pill use, iron therapy or curettage	No or Male Yes	0 points 1 point
Requiring treatment with blood transfusion or replacement therapy or desmopressin	No or Male Yes	0 points 2 points
Requiring treatment with hysterectomy	No or Male Yes	0 points 3 points
Menorrhagia Index Score [Meno_In] [Maximum Sum: 11 points]

Postpartum Bleeding
Postpartum bleeding	Never delivered or No or Male Yes	0 points -1 points 1 point
Frequency >1 time	Never delivered or No or Male Yes	0 points 0 points 1 point
Spontaneous5	Never delivered or No or Male Yes	0 points 0 points 1 point
On prophylaxis	Never delivered or No or male Yes	0 points 0 points 1 point
Causing anaemia	Never delivered or No or Male Yes	0 points 0 points 1 point
Requiring treatment with antifibrinolytics, iron therapy or curettage	Never delivered or No or Male Yes	0 points 0 points 1 point
Requiring treatment with blood transfusion or replacement therapy or desmopressin	Never delivered or No or Male Yes	0 points 0 points 2 points
Requiring treatment with hysterectomy	Never delivered or No or Male Yes	0 points 0 points 3 points
Postpartum Bleeding Index Score [PPH_In] [Maximum Sum: 11 points]

⁵Postpartum bleeding/haemorrhage should be considered as spontaneous when it is not associated with an identified gynaecological or obstetrical cause for primary postpartum haemorrhage [PPH].

Bleeding during Tooth Extraction
Bleeding during tooth extraction	Never done No Yes	0 points -1 points 1 point
Frequency > 1 time	Never done No Yes	0 points 0 points 1 point
On prophylaxis	Never done No Yes	0 points 0 points 1 point
For ≥ 15 min	Never done No Yes	0 points 0 points 1 point
Recurrence of bleeding	Never done No Yes	0 points 0 points 1 point
Requiring treatment with resuturing or packing or antifibrinolytics	Never done No Yes	0 points 0 points 1 point
Requiring treatment with blood transfusion or replacement therapy or desmopressin	Never done No Yes	0 points 0 points 2 points
Tooth Extraction Bleeding Index Score [Tooth_In] [Maximum Sum: 8 points]

Bleeding during Minor Surgery6
Bleeding during minor surgery	Never done No Yes	0 points -1 points 1 point
Frequency > 1 time	Never done No Yes	0 points 0 points 1 point
On prophylaxis	Never done No Yes	0 points 0 points 1 point
Postoperative bleeding	Never done No Yes	0 points 0 points 1 point
Requiring treatment with resuturing or packing or antifibrinolytics	Never done No Yes	0 points 0 points 1 point
Requiring treatment with blood transfusion or replacement therapy or desmopressin	Never done No Yes	0 points 0 points 2 points
Minor Surgery Bleeding Index Score [Minor_In] [Maximum Sum:7 points]

⁶See References for the definition of Major and Minor surgery.

Bleeding during Tonsillectomy7
Bleeding during tonsillectomy	Never done No Yes	0 points -1 points 1 point
On prophylaxis	Never done No Yes	0 points 0 points 1 point
Postoperative bleeding	Never done No Yes	0 points 0 points 1 point
Requiring treatment with surgical hemostasis or antifibrinolytics	Never done No Yes	0 points 0 points 1 point
Requiring treatment with blood transfusion or replacement therapy or desmopressin	Never done No Yes	0 points 0 points 1 point
Tonsillectomy Bleeding Index Score [Tonsil_In] [Maximum Sum: 5 points]

⁷With or without adenoidectomy

Bleeding during Major Surgery6
Bleeding during major surgery	Never done No Yes	0 points -1 points 1 point
Frequency >1 time	Never done No Yes	0 points 0 points 1 point
On prophylaxis	Never done No Yes	0 points 0 points 1 point
Postoperative bleeding	Never done No Yes	0 points 0 points 1 point
Requiring treatment with surgical hemostasis or antifibrinolytics	Never done No Yes	0 points 0 points 1 point
Requiring treatment with blood transfusion or replacement therapy or desmopressin	Never done No Yes	0 points 0 points 2 points
Major Surgery Bleeding Index Score [Major_In] [Maximum Sum: 7 points]

⁶See References for the definition of Major and Minor surgery.

Gender
Female Male		0 points 1 point
Gender Score

Age
Age [Years]

Total Score [Total_Score]

Bleeding Score

RBD Probability [%]

The algorithm was able to differentiate patients with a RBD from healthy individuals. A bleeding score value of 1.5 had the highest sensitivity (67.1%) and specificity (73.8%) in discriminating patients with a RBD from those without.

For additional information on the analysis of data - see References.

Click HERE to re-set the EN-RBD-BS algorithm.

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