Table 3 Open in new tab Rare bleeding...

Prevalence

Bleeding phenotype

Risk of thrombosis

Fibrinogen—Factor I
Fibrinogen deficiency is complex: one distinguishes between afibrinogenaemia, hypofibrinogenaemia and dysfibrinogenaemia. Patients with dysfibrinogenaemia may have an elevated thrombotic risk and no bleeding risk.

1:1000000

Usually mild and heterogenous even in afibrinogenemia

Severe thromboembolic events might occur: both venous and arterial, independent of age.

Prothrombin—Factor II

1:2000000

Moderate to severe (when factor levels are low)

Non reported

Factor V
Factor V Leiden does not lead to factor V deficiency and is different form the bleeding disorder ‘Factor V-deficiency’.

1:1000000

Usually mild to moderate depending on factor level

Severe thromboembolic events might occur: both venous and arterial, independent of age.

Combined factor V + VIII

1:1000000

Usually mild to moderate depending on factor levels

Severe thromboembolic events might occur: both venous and arterial, independent of age.

Factor VII

1:500000

Mild to severe (no proportional correlation to factor level)

Severe thromboembolic events might occur: both venous and arterial, independent of age.

Factor X

1:1000000

Moderate to severe depending on factor level

Not reported.

Factor XI

1:1000000

Usually mild and mainly soft tissue bleeding

Severe thromboembolic events might occur: both venous and arterial, independent of age.

Factor XIII

1:2000000

Moderate to severe depending on factor level

Severe thromboembolic events might occur: both venous and arterial, independent of age.

Table 3

Open in new tab

Rare bleeding disorders and associated factor deficiencies

	Prevalence	Bleeding phenotype	Risk of thrombosis
Fibrinogen—Factor I Fibrinogen deficiency is complex: one distinguishes between afibrinogenaemia, hypofibrinogenaemia and dysfibrinogenaemia. Patients with dysfibrinogenaemia may have an elevated thrombotic risk and no bleeding risk.	1:1000000	Usually mild and heterogenous even in afibrinogenemia	Severe thromboembolic events might occur: both venous and arterial, independent of age.
Prothrombin—Factor II	1:2000000	Moderate to severe (when factor levels are low)	Non reported
Factor V Factor V Leiden does not lead to factor V deficiency and is different form the bleeding disorder ‘Factor V-deficiency’.	1:1000000	Usually mild to moderate depending on factor level	Severe thromboembolic events might occur: both venous and arterial, independent of age.
Combined factor V + VIII	1:1000000	Usually mild to moderate depending on factor levels	Severe thromboembolic events might occur: both venous and arterial, independent of age.
Factor VII	1:500000	Mild to severe (no proportional correlation to factor level)	Severe thromboembolic events might occur: both venous and arterial, independent of age.
Factor X	1:1000000	Moderate to severe depending on factor level	Not reported.
Factor XI	1:1000000	Usually mild and mainly soft tissue bleeding	Severe thromboembolic events might occur: both venous and arterial, independent of age.
Factor XIII	1:2000000	Moderate to severe depending on factor level	Severe thromboembolic events might occur: both venous and arterial, independent of age.

	Prevalence	Bleeding phenotype	Risk of thrombosis
Fibrinogen—Factor I Fibrinogen deficiency is complex: one distinguishes between afibrinogenaemia, hypofibrinogenaemia and dysfibrinogenaemia. Patients with dysfibrinogenaemia may have an elevated thrombotic risk and no bleeding risk.	1:1000000	Usually mild and heterogenous even in afibrinogenemia	Severe thromboembolic events might occur: both venous and arterial, independent of age.
Prothrombin—Factor II	1:2000000	Moderate to severe (when factor levels are low)	Non reported
Factor V Factor V Leiden does not lead to factor V deficiency and is different form the bleeding disorder ‘Factor V-deficiency’.	1:1000000	Usually mild to moderate depending on factor level	Severe thromboembolic events might occur: both venous and arterial, independent of age.
Combined factor V + VIII	1:1000000	Usually mild to moderate depending on factor levels	Severe thromboembolic events might occur: both venous and arterial, independent of age.
Factor VII	1:500000	Mild to severe (no proportional correlation to factor level)	Severe thromboembolic events might occur: both venous and arterial, independent of age.
Factor X	1:1000000	Moderate to severe depending on factor level	Not reported.
Factor XI	1:1000000	Usually mild and mainly soft tissue bleeding	Severe thromboembolic events might occur: both venous and arterial, independent of age.
Factor XIII	1:2000000	Moderate to severe depending on factor level	Severe thromboembolic events might occur: both venous and arterial, independent of age.

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