Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

INITIAL

VWD type unknown with active severe haemorrhage

1st line – von Willebrand factor (VWF)-containing concentrate

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INITIAL
|
VWD type unknown with active severe haemorrhage
1st line – 

von Willebrand factor (VWF)-containing concentrate

Patients with active severe haemorrhage require resuscitation as appropriate, along with treatments specific for VWD.

These patients should be treated as if they have type 3 VWD until more historical and/or laboratory information allows identification of the specific type of VWD.

Most VWF concentrates contain both VWF and factor VIII. However, in patients given high-purity VWF, it takes approximately 6-8 hours for the endogenous levels of factor VIII to reach haemostatic levels.[3] Therefore, an initial loading dose of factor VIII must be given when bleeding is treated with high-purity plasma VWF concentrate or recombinant VWF concentrate.

VWF-containing concentrates may differ in the amount of factor VIII, the VWF:factor VIII ratio, and the multimeric composition of VWF. At present, most available products are plasma-derived and undergo treatment to minimise the risk of viral transmission. Recombinant VWF is also available.

VWF and factor VIII levels should be monitored if VWF concentrate is administered repeatedly. Dosing should be adjusted such that factor VIII levels are not excessively elevated because of a potential risk of thrombosis.[25]

Pregnant women with severe haemorrhage should generally receive the same treatment as non-pregnant women. There are no data on the long-term administration of tranexamic acid in pregnancy, but it is known to cross the placenta. Antenatal care should be provided in a specialist centre by a multidisciplinary team, including haemotologists and obstetricians with expertise in this field.

Consider – platelet transfusion

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INITIAL
|
VWD type unknown with active severe haemorrhage
Consider – 

platelet transfusion

Additional treatment recommended for SOME patients in selected patient group

Platelet transfusion may be useful in the rare patient with continued bleeding despite treatment with von Willebrand factor-containing concentrates.[20]

2nd line – cryoprecipitate

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INITIAL
|
VWD type unknown with active severe haemorrhage
2nd line – 

cryoprecipitate

Patients with active severe haemorrhage require resuscitation as appropriate, along with treatments specific for VWD.

Except in an emergency, cryoprecipitate should be avoided because it does not undergo viral inactivation. In most countries, virally inactivated von Willebrand factor concentrate is available and should be used in preference to cryoprecipitate.

Consider – platelet transfusion

Back
INITIAL
|
VWD type unknown with active severe haemorrhage
Consider – 

platelet transfusion

Additional treatment recommended for SOME patients in selected patient group

Platelet transfusion may be useful in the rare patient with continued bleeding despite treatment with cryoprecipitate.[20]

all types VWD with severe bleeding uncontrolled by desmopressin, antifibrinolytics, and von Willebrand factor-containing concentrates

1st line – platelet transfusion

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INITIAL
|
all types VWD with severe bleeding uncontrolled by desmopressin, antifibrinolytics, and von Willebrand factor-containing concentrates
1st line – 

platelet transfusion

In patients with bleeding refractory to plasma replacement therapy, platelets are a useful source of von Willebrand factor (VWF), which is released at the site of injury. Platelet VWF is absent in patients with type 3 VWD.

Antenatal care should be provided in a specialist centre by a multidisciplinary team, including haemotologists and obstetricians with expertise in this field

2nd line – cryoprecipitate

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INITIAL
|
all types VWD with severe bleeding uncontrolled by desmopressin, antifibrinolytics, and von Willebrand factor-containing concentrates
2nd line – 

cryoprecipitate

Used as for factor concentrate, but carries greater risk of viral transmission.

ACUTE

all types VWD with severe bleeding or before high-risk bleeding procedures (including where sustained high levels of VWF required for several days): with or without mucosal involvement

1st line – VWF-containing concentrate

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ACUTE
|
all types VWD with severe bleeding or before high-risk bleeding procedures (including where sustained high levels of VWF required for several days): with or without mucosal involvement
1st line – 

VWF-containing concentrate

In patients with severe bleeding or undergoing surgical procedures where any increase in bleeding carries risk, such as a neurosurgical procedure and/or when sustained levels of von Willebrand factor (VWF) are needed for several days, a VWF-containing concentrate should be used.

Mucosal involvement includes the gastrointestinal tract, mouth, and genitourinary tract, while non-mucosal involvement excludes these sites.

Most VWF concentrates contain both VWF and factor VIII. However, in patients given high-purity VWF, it takes approximately 6-8 hours for the endogenous levels of factor VIII to reach haemostatic levels.[3] Therefore, an initial loading dose of factor VIII must be given when bleeding is treated with high-purity plasma VWF concentrate or recombinant VWF concentrate.

VWF-containing concentrates may differ in the amount of factor VIII, the VWF:factor VIII ratio, and the multimeric composition of VWF. At present, most available products are plasma-derived and undergo treatment to minimise the risk of viral transmission. Recombinant VWF is also available.

VWF and factor VIII levels should be monitored if VWF concentrate is administered repeatedly. Dosing should be adjusted such that factor VIII levels are not excessively elevated because of a potential risk of thrombosis.[25]

Pregnant women with severe hemorrhage should generally receive the same treatment as non-pregnant women. There are no data on the long-term administration of tranexamic acid in pregnancy, but it is known to cross the placenta. Antenatal care should be provided in a specialist centre by a multidisciplinary team, including haemotologists and obstetricians with expertise in this field.

Consider – antifibrinolytic therapy

Back
ACUTE
|
all types VWD with severe bleeding or before high-risk bleeding procedures (including where sustained high levels of VWF required for several days): with or without mucosal involvement
Consider – 

antifibrinolytic therapy

Additional treatment recommended for SOME patients in selected patient group

Tranexamic acid and aminocaproic acid should not be used in upper renal tract bleeding or in the presence of disseminated intravascular coagulation.

One dose before dental cleanings may be effective to prevent oozing. Repeat dosing may be effective alone to treat bleeding from mucosal surfaces.

May be used in combination with factor concentrate for mucous membrane procedures (i.e., involving the gastrointestinal tract, mouth, or genitourinary tract) or non-mucous membrane procedures.

Aminocaproic acid and tranexamic acid may be given orally or intravenously. The choice depends on patient factors and convenience. For example, the intravenous route will mostly be used for patients undergoing surgery.

If available, tranexamic acid mouthwash is also extremely useful for bleeding in the oral cavity. It can be swallowed or not, as preferred. The mouthwash may be prepared by a pharmacist.

Primary options

tranexamic acid: children and adults: 10 mg/kg intravenously immediately before procedure, followed by 10 mg/kg three to four times daily for 2-8 days; adults: 1 to 1.5 g orally two to three times daily

OR

aminocaproic acid: adults: 5 g orally/intravenously initially as a loading dose in the first hour, followed by 1 g/hour for 8 hours or until response, maximum 30 g/day

all types VWD with minor bleeding or before procedures: involving mucous membranes

1st line – antifibrinolytic therapy

Back
ACUTE
|
all types VWD with minor bleeding or before procedures: involving mucous membranes
1st line – 

antifibrinolytic therapy

Tranexamic acid and aminocaproic acid should not be used in upper renal tract bleeding or in the presence of disseminated intravascular coagulation.

One dose before dental cleanings may be effective to prevent oozing. Repeat dosing may be effective alone to treat bleeding from mucosal surfaces.

Used in combination with desmopressin or factor concentrate for mucous membrane procedures (i.e., involving the gastrointestinal tract, mouth, or genitourinary tract).

Aminocaproic acid and tranexamic acid may be given orally or intravenously. The choice between the two routes depends on patient factors and convenience. For example, the intravenous route will mostly be used for patients undergoing surgery.

If available, tranexamic acid mouthwash is also extremely useful for bleeding in the oral cavity. It can be swallowed or not, as preferred. The mouthwash may be prepared by a pharmacist.

Pregnant women should receive the same treatment as non-pregnant women. There are no data on the long-term administration of tranexamic acid in pregnancy, but it is known to cross the placenta. Antenatal care should be provided in a specialist centre by a multidisciplinary team, including haemotologists and obstetricians with expertise in this field.

Primary options

tranexamic acid: children and adults: 10 mg/kg intravenously immediately before procedure, followed by 10 mg/kg three to four times daily for 2-8 days; adults: 1 to 1.5 g orally two to three times daily

OR

aminocaproic acid: adults: 5 g orally/intravenously initially as a loading dose in the first hour, followed by 1 g/hour for 8 hours or until response, maximum 30 g/day

Plus – desmopressin

Back
ACUTE
|
all types VWD with minor bleeding or before procedures: involving mucous membranes
|
known desmopressin responder
Plus – 

desmopressin

Treatment recommended for ALL patients in selected patient group

Desmopressin may be used as an adjunct to antifibrinolytic therapy in patients known to respond to this drug.

All patients with type 1, 2A, or 2M VWD should be tested to see whether they respond to desmopressin, unless its use is contra-indicated (e.g., patients with atherosclerosis, cardiac insufficiency or other conditions that are treated with diuretics, psychogenic polydipsia, and polydipsia in alcohol dependence).

Desmopressin is generally ineffective in type 2B (although its use in this setting has been reported) and it may worsen thrombocytopenia. Desmopressin is ineffective in type 2N, as it results in the release of a defective protein.

Patients should be tested to assess response >1 week before any planned treatment. The test dose is the same as a single treatment dose of desmopressin.

The intravenous or subcutaneous route is preferred for in-patient treatment and surgery, while the intranasal route is used to allow patients to self-treat at home.

Patients should have at least a 3- to 5-fold increase in factor VIII and von Willebrand factor (VWF) 30 minutes to 1 hour after intravenous or subcutaneous administration and 1 hour after intranasal administration, with resulting values within the normal range. Some patients with type 1 VWD have accelerated clearance of VWF, and so a fall-off measurement should also be obtained at 4-6 hours after administration.

Tachyphylaxis occurs after repeat dosing; desmopressin is generally not given for >3 days because of this.

Primary options

desmopressin: children >2 years of age and adults: 0.3 micrograms/kg/dose intravenously/subcutaneously 30 minutes before procedure, may repeat every 12-24 hours according to response

OR

desmopressin nasal: children >2 years of age and adults <50 kg body weight: (1.5 mg/mL) 150 micrograms in one nostril 2 hours before procedure, may repeat every 12-24 hours according to response; adults >50 kg body weight: (1.5 mg/mL) 150 micrograms in both nostrils 2 hours before procedure, may repeat every 12-24 hours according to response

Plus – VWF-containing concentrate

Back
ACUTE
|
all types VWD with minor bleeding or before procedures: involving mucous membranes
|
known desmopressin non-responder
Plus – 

VWF-containing concentrate

Treatment recommended for ALL patients in selected patient group

Von Willebrand factor (VWF)-containing concentrate can be used in: patients with type 1, 2A, or 2M VWD who do not respond to desmopressin; patients with type 3, 2B, or 2N VWD; and patients who have contra-indications to desmopressin use (this includes those with atherosclerosis, cardiac insufficiency or other conditions that are treated with diuretics, psychogenic polydipsia, and polydipsia in alcohol dependence).

Most VWF concentrates contain both VWF and factor VIII. However, in patients given high-purity VWF, it takes approximately 6-8 hours for the endogenous levels of factor VIII to reach haemostatic levels.[3] Therefore, an initial loading dose of factor VIII must be given when bleeding is treated with high-purity plasma VWF concentrate or recombinant VWF concentrate.

VWF-containing concentrates may differ in the amount of factor VIII, the VWF:factor VIII ratio, and the multimeric composition of VWF. At present, most available products are plasma-derived and undergo treatment to minimise the risk of viral transmission. Recombinant VWF is also available.

VWF and factor VIII levels should be monitored if VWF concentrate is administered repeatedly. Dosing should be adjusted such that factor VIII levels are not excessively elevated because of a potential risk of thrombosis.[25]

type 1, 2A, or 2M VWD: desmopressin responder with moderate mucosal or minor/moderate non-mucosal bleeding or before procedures not involving mucous membranes: non-pregnant

1st line – desmopressin

Back
ACUTE
|
type 1, 2A, or 2M VWD: desmopressin responder with moderate mucosal or minor/moderate non-mucosal bleeding or before procedures not involving mucous membranes: non-pregnant
1st line – 

desmopressin

All patients with type 1, 2A, or 2M VWD should be tested to see whether they respond to desmopressin, unless its use is contra-indicated (e.g., patients with atherosclerosis, cardiac insufficiency or other conditions that are treated with diuretics, psychogenic polydipsia, and polydipsia in alcohol dependence).

Mucosal involvement includes the gastrointestinal tract, mouth, and genitourinary tract.

Desmopressin is generally ineffective in type 2B (although its use in this setting has been reported) and it may worsen thrombocytopenia. Desmopressin is ineffective in type 2N, as it results in the release of a defective protein.

Patients should be tested to assess respose >1 week before any planned treatment. The test dose is the same as a single treatment dose of desmopressin.

The intravenous or subcutaneous route is preferred for inpatient treatment and surgery, while the intranasal route is used to allow patients to self-treat at home.

Patients should have at least a 3- to 5-fold increase in factor VIII and von Willebrand factor 30 minutes to 1 hour after intravenous or subcutaneous administration and 1 hour after intranasal administration, with resulting values within the normal range. Some patients with type 1 VWD have accelerated clearance of von Willebrand factor, and so a fall-off measurement should also be obtained at 4-6 hours after administration.

Tachyphylaxis may occur after repeat dosing: desmopressin is generally not given for >3 days because of this (but can be continued according to measured response).

Primary options

desmopressin: children >2 years of age and adults: 0.3 micrograms/kg/dose intravenously/subcutaneously 30 minutes before procedure, may repeat every 12-24 hours according to response

OR

desmopressin nasal: children >2 years of age and adults <50 kg body weight: (1.5 mg/mL) 150 micrograms in one nostril 2 hours before procedure, may repeat every 12-24 hours according to response; adults >50 kg body weight: (1.5 mg/mL) 150 micrograms in both nostrils 2 hours before procedure, may repeat every 12-24 hours according to response

Consider – antifibrinolytic therapy

Back
ACUTE
|
type 1, 2A, or 2M VWD: desmopressin responder with moderate mucosal or minor/moderate non-mucosal bleeding or before procedures not involving mucous membranes: non-pregnant
Consider – 

antifibrinolytic therapy

Additional treatment recommended for SOME patients in selected patient group

Tranexamic acid and aminocaproic acid should not be used in upper renal tract bleeding or in the presence of disseminated intravascular coagulation.

One dose before dental cleanings may be effective to prevent oozing. Repeat dosing may be effective alone to treat bleeding from mucosal surfaces.

May be used in combination with desmopressin or factor concentrate for mucous membrane procedures (i.e., involving the gastrointestinal tract, mouth, or genitourinary tract) or non-mucous membrane procedures.

Aminocaproic acid and tranexamic acid may be given orally or intravenously. The choice depends on patient factors and convenience. For example, the intravenous route will mostly be used for patients undergoing surgery.

If available, tranexamic acid mouthwash is also extremely useful for bleeding in the oral cavity. It can be swallowed or not, as preferred. The mouthwash may be prepared by a pharmacist.

Primary options

tranexamic acid: children and adults: 10 mg/kg intravenously immediately before procedure, followed by 10 mg/kg three to four times daily for 2-8 days; adults: 1 to 1.5 g orally two to three times daily

OR

aminocaproic acid: adults: 5 g orally/intravenously initially as a loading dose in the first hour, followed by 1 g/hour for 8 hours or until response, maximum 30 g/day

2nd line – VWF-containing concentrate

Back
ACUTE
|
type 1, 2A, or 2M VWD: desmopressin responder with moderate mucosal or minor/moderate non-mucosal bleeding or before procedures not involving mucous membranes: non-pregnant
2nd line – 

VWF-containing concentrate

When desmopressin is insufficient to control bleeding, von Willebrand factor (VWF)-containing concentrates are required.

Mucosal involvement includes the gastrointestinal tract, mouth, and genitourinary tract.

Most VWF concentrates contain both VWF and factor VIII. However, in patients given high-purity VWF, it takes approximately 6-8 hours for the endogenous levels of factor VIII to reach haemostatic levels.[3] Therefore, an initial loading dose of factor VIII must be given when bleeding is treated with high-purity plasma VWF concentrate or recombinant VWF concentrate.

VWF-containing concentrates may differ in the amount of factor VIII, the VWF:factor VIII ratio, and the multimeric composition of VWF. At present, most available products are plasma-derived and undergo treatment to minimise the risk of viral transmission.

Recombinant VWF is also available.

VWF and factor VIII levels should be monitored if VWF concentrate is administered repeatedly. Dosing should be adjusted such that factor VIII levels are not excessively elevated because of a potential risk of thrombosis.[25]

Consider – antifibrinolytic therapy

Back
ACUTE
|
type 1, 2A, or 2M VWD: desmopressin responder with moderate mucosal or minor/moderate non-mucosal bleeding or before procedures not involving mucous membranes: non-pregnant
Consider – 

antifibrinolytic therapy

Additional treatment recommended for SOME patients in selected patient group

Tranexamic acid and aminocaproic acid should not be used in upper renal tract bleeding or in the presence of disseminated intravascular coagulation.

One dose before dental cleanings may be effective to prevent oozing. Repeat dosing may be effective alone to treat bleeding from mucosal surfaces.

May be used in combination with desmopressin or factor concentrate for mucous membrane procedures (i.e., involving the gastrointestinal tract, mouth, or genitourinary tract) or non-mucous membrane procedures.

Aminocaproic acid and tranexamic acid may be given orally or intravenously. The choice depends on patient factors and convenience. For example, the intravenous route will mostly be used for patients undergoing surgery.

If available, tranexamic acid mouthwash is also extremely useful for bleeding in the oral cavity. It can be swallowed or not, as preferred. The mouthwash may be prepared by a pharmacist.

Primary options

tranexamic acid: children and adults: 10 mg/kg intravenously immediately before procedure, followed by 10 mg/kg three to four times daily for 2-8 days; adults: 1 to 1.5 g orally two to three times daily

OR

aminocaproic acid: adults: 5 g orally/intravenously initially as a loading dose in the first hour, followed by 1 g/hour for 8 hours or until response, maximum 30 g/day

type 1, 2A, or 2M VWD: desmopressin non-responder with moderate mucosal or minor/moderate non-mucosal bleeding or before procedures not involving mucous membranes: non-pregnant

1st line – VWF-containing concentrate

Back
ACUTE
|
type 1, 2A, or 2M VWD: desmopressin non-responder with moderate mucosal or minor/moderate non-mucosal bleeding or before procedures not involving mucous membranes: non-pregnant
1st line – 

VWF-containing concentrate

Von Willebrand factor (VWF)-containing concentrate may be used in desmopressin non-responders or patients with contraindications to desmopressin (this includes those with atherosclerosis, cardiac insufficiency or other conditions that are treated with diuretics, psychogenic polydipsia, and polydipsia in alcohol dependence).

Mucosal involvement includes the gastrointestinal tract, mouth, and genitourinary tract.

Most VWF concentrates contain both VWF and factor VIII. However, in patients given high-purity VWF, it takes approximately 6-8 hours for the endogenous levels of factor VIII to reach haemostatic levels.[3] Therefore, an initial loading dose of factor VIII must be given when bleeding is treated with high-purity plasma VWF concentrate or recombinant VWF concentrate.

VWF-containing concentrates may differ in the amount of factor VIII, the VWF:factor VIII ratio, and the multimeric composition of VWF. At present, most available products are plasma-derived and undergo treatment to minimise the risk of viral transmission. Recombinant VWF is also available.

VWF and factor VIII levels should be monitored if VWF concentrate is administered repeatedly. Dosing should be adjusted such that factor VIII levels are not excessively elevated because of a potential risk of thrombosis.[25]

Consider – antifibrinolytic therapy

Back
ACUTE
|
type 1, 2A, or 2M VWD: desmopressin non-responder with moderate mucosal or minor/moderate non-mucosal bleeding or before procedures not involving mucous membranes: non-pregnant
Consider – 

antifibrinolytic therapy

Additional treatment recommended for SOME patients in selected patient group

Tranexamic acid and aminocaproic acid should not be used in upper renal tract bleeding or in the presence of disseminated intravascular coagulation.

One dose before dental cleanings may be effective to prevent oozing. Repeat dosing may be effective alone to treat bleeding from mucosal surfaces.

May be used in combination with desmopressin or factor concentrate for mucous membrane procedures (i.e., involving the gastrointestinal tract, mouth, or genitourinary tract) or non-mucous membrane procedures.

Aminocaproic acid and tranexamic acid may be given orally or intravenously. The choice depends on patient factors and convenience. For example, the intravenous route will mostly be used for patients undergoing surgery.

If available, tranexamic acid mouthwash is also extremely useful for bleeding in the oral cavity. It can be swallowed or not, as preferred. The mouthwash may be prepared by a pharmacist.

Primary options

tranexamic acid: children and adults: 10 mg/kg intravenously immediately before procedure, followed by 10 mg/kg three to four times daily for 2-8 days; adults: 1 to 1.5 g orally two to three times daily

OR

aminocaproic acid: adults: 5 g orally/intravenously initially as a loading dose in the first hour, followed by 1 g/hour for 8 hours or until response, maximum 30 g/day

type 3, 2B, or 2N VWD: all bleeding or procedures except minor mucosal bleeding or before minor procedures involving mucosal surfaces: non-pregnant

1st line – VWF-containing concentrate

Back
ACUTE
|
type 3, 2B, or 2N VWD: all bleeding or procedures except minor mucosal bleeding or before minor procedures involving mucosal surfaces: non-pregnant
1st line – 

VWF-containing concentrate

Patients with type 3 VWD generally require treatment with von Willebrand factor (VWF)-containing concentrates.[3][20] Except in an emergency, cryoprecipitate should be avoided because it does not undergo viral inactivation. In most countries a virally inactivated VWF concentrate is available.

Most VWF concentrates contain both VWF and factor VIII. However, in patients given high-purity VWF, it takes approximately 6-8 hours for the endogenous levels of factor VIII to reach haemostatic levels.[3] Therefore, an initial loading dose of factor VIII must be given when bleeding is treated with high-purity plasma VWF concentrate or recombinant VWF concentrate.

VWF-containing concentrates may differ in the amount of factor VIII, the VWF:factor VIII ratio, and the multimeric composition of VWF. At present, most available products are plasma-derived and undergo treatment to minimise the risk of viral transmission. Recombinant VWF is also available.

Aggressive dosing is needed for type 3 VWD. For serious bleeding and major surgery, laboratory monitoring of VWF levels is necessary and should be managed by a haematologist with expertise in bleeding disorders.

Consider – antifibrinolytic therapy

Back
ACUTE
|
type 3, 2B, or 2N VWD: all bleeding or procedures except minor mucosal bleeding or before minor procedures involving mucosal surfaces: non-pregnant
Consider – 

antifibrinolytic therapy

Additional treatment recommended for SOME patients in selected patient group

Tranexamic acid and aminocaproic acid should not be used in upper renal tract bleeding or in the presence of disseminated intravascular coagulation.

One dose before dental cleanings may be effective to prevent oozing. Repeat dosing may be effective alone to treat bleeding from mucosal surfaces.

May be used in combination with factor concentrate for mucous membrane procedures (i.e., involving the gastrointestinal tract, mouth, or genitourinary tract) or non-mucous membrane procedures.

Aminocaproic acid and tranexamic acid may be given orally or intravenously. The choice depends on patient factors and convenience. For example, the intravenous route will mostly be used for patients undergoing surgery.

If available, tranexamic acid mouthwash is also extremely useful for bleeding in the oral cavity. It can be swallowed or not, as preferred. The mouthwash may be prepared by a pharmacist.

Primary options

tranexamic acid: children and adults: 10 mg/kg intravenously immediately before procedure, followed by 10 mg/kg three to four times daily for 2-8 days; adults: 1 to 1.5 g orally two to three times daily

OR

aminocaproic acid: adults: 5 g orally/intravenously initially as a loading dose in the first hour, followed by 1 g/hour for 8 hours or until response, maximum 30 g/day

all types VWD: pregnant

1st line – VWF-containing concentrate

Back
ACUTE
|
all types VWD: pregnant
1st line – 

VWF-containing concentrate

Antenatal care should be provided in a specialist centre by a multidisciplinary team, including haemotologists and obstetricians with expertise in this field.

In type 1 VWD, especially if mild, individuals may experience a significant rise in von Willebrand factor (VWF) level. This may result in VWF levels that are within the normal range at term, so that no treatment or special measures are required. If VWF activity does not rise to normal, VWF-containing concentrate should be used.[2] 

In type 2 VWD, although VWF levels may rise during pregnancy, the functional activity will rarely enter the normal range and VWF replacement will be necessary.[2]

In type 3 VWD, there is no rise in VWF during pregnancy and VWF replacement is required for delivery and procedures. Replacement during gestation is not usually required, unless there is bleeding or an additional risk factor.[2]

2nd line – desmopressin

Back
ACUTE
|
all types VWD: pregnant
2nd line – 

desmopressin

If von Willebrand factor (VWF) activity does not rise to normal and the patient is responsive to desmopressin (type 1 and some type 2), this treatment can be used as an alternative to VWF-containing concentrate.[2]Desmopressin is safe to administer during pregnancy but should not be used in the presence of pre-eclampsia.

Primary options

desmopressin: 0.3 micrograms/kg/dose intravenously/subcutaneously 30 minutes before procedure, may repeat every 12-24 hours according to response

OR

desmopressin nasal: <50 kg body weight: (1.5 mg/mL) 150 micrograms in one nostril 2 hours before procedure, may repeat every 12-24 hours according to response; >50 kg body weight: (1.5 mg/mL) 150 micrograms in both nostrils 2 hours before procedure, may repeat every 12-24 hours according to response

Plus – platelet transfusion

Back
ACUTE
|
all types VWD: pregnant
|
with thrombocytopenia
Plus – 

platelet transfusion

Treatment recommended for ALL patients in selected patient group

Thrombocytopenia can worsen with pregnancy, and platelet transfusion may be needed for delivery.

ONGOING

all types VWD with chronic or recurrent menorrhagia

1st line – oral hormonal therapy

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ONGOING
|
all types VWD with chronic or recurrent menorrhagia
1st line – 

oral hormonal therapy

Used to treat menorrhagia where benefits of treatment outweigh any risks. Oestrogen-containing compounds may also be effective in refractory gastrointestinal bleeding, but evidence is anecdotal.

For menorrhagia, combined oestrogen/progestogen and progesterone-only contraceptives usually decrease menstrual blood flow.

1st line – antifibrinolytic therapy

Back
ONGOING
|
all types VWD with chronic or recurrent menorrhagia
1st line – 

antifibrinolytic therapy

Documented to be effective in treatment of menorrhagia in women without bleeding disorders. The efficacy of aminocaproic acid in treating menorrhagia is assumed from studies using tranexamic acid; lower dose may be effective and better tolerated.

Primary options

tranexamic acid: adults: 1 to 1.5 g orally two to three times daily on the first 2-3 days of menses

OR

aminocaproic acid: adults: doses of 2-6 g orally every 6 hours on the first 5 days of menses have been reported; however, consult specialist for further guidance on dose

2nd line – desmopressin

Back
ONGOING
|
all types VWD with chronic or recurrent menorrhagia
2nd line – 

desmopressin

Desmopressin is an alternative therapy for menorrhagia for those who do not respond to, or cannot tolerate, other measures. For home therapy this is most readily given as an intranasal spray. Occasionally, patients do not respond to intranasal therapy but do respond to subcutaneous desmopressin, which can also be self-administered at home.

Case reports indicate that desmopressin is safe and effective in pregnancy.[26]

Primary options

desmopressin nasal: (1.5 mg/mL) 150 micrograms in both nostrils once or twice daily for first 1-2 days of bleeding

OR

desmopressin: 0.3 micrograms/kg/dose subcutaneously given at onset of bleeding, may repeat every 12 hours according to response; maximum of 3 doses

2nd line – intrauterine device

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ONGOING
|
all types VWD with chronic or recurrent menorrhagia
2nd line – 

intrauterine device

Although evidence in women with VWD is sparse, the progestogen-releasing intrauterine device has been shown to be effective in treatment of menorrhagia in women without bleeding disorders.

Primary options

levonorgestrel intrauterine device: 52 mg/device every 5 years

all types VWD with significant chronic or recurrent bleeding or with treatment-refractory menorrhagia

1st line – prophylaxis with VWF-containing concentrate

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ONGOING
|
all types VWD with significant chronic or recurrent bleeding or with treatment-refractory menorrhagia
1st line – 

prophylaxis with VWF-containing concentrate

Prevents bleeding in patients who have had recurrent bleeding.

Patients and/or family members can be instructed in home infusion. Patients with type 3 VWD can occasionally develop antibodies to von Willebrand factor (VWF) after treatment with VWF.

VWF-containing concentrates may differ in the amount of factor VIII, the VWF:factor VIII ratio, and the multimeric composition of VWF. At present, most available products are plasma-derived and undergo treatment to minimise the risk of viral transmission. Recombinant VWF is also available.

VWF and factor VIII levels should be monitored if VWF concentrate is administered repeatedly. Dosing should be adjusted such that factor VIII levels are not excessively elevated because of a potential risk of thrombosis.[25]

Pregnant women should receive the same treatment as non-pregnant women. There are no data on the long-term administration of tranexamic acid in pregnancy, but it is known to cross the placenta. Antenatal care should be provided in a specialist centre by a multidisciplinary team, including haemotologists and obstetricians with expertise in this field.

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Choose a patient group to see our recommendations

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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