Von Willebrand Disease
Clinical Snapshot
Von Willebrand disease (VWD) is the most common inherited bleeding disorder, affecting an estimated 1% of the population by laboratory criteria, though far fewer have clinically significant bleeding. It results from a quantitative deficiency or qualitative dysfunction of von Willebrand factor (VWF), a large multimeric glycoprotein with two distinct hemostatic roles: it mediates platelet adhesion to exposed subendothelial collagen at a site of vascular injury (primary hemostasis), and it binds and stabilizes circulating factor VIII (FVIII) in plasma, protecting it from premature clearance (secondary hemostasis). VWD therefore produces a mixed bleeding phenotype: mucocutaneous bleeding — epistaxis, easy bruising, gum bleeding, and heavy menstrual bleeding — dominates in most patients, while more severe disease, particularly type 3 and type 2N, adds a secondary FVIII deficiency capable of producing hemophilia-like deep muscle and joint bleeding.[1]
VWD is classified into 3 principal types that directly determine treatment selection rather than merely describing severity: type 1 is a partial quantitative deficiency (roughly 75% of diagnosed cases, generally mild-to-moderate); type 2 is a qualitative dysfunction with 4 distinct subtypes (2A, 2B, 2M, 2N) that behave and are treated differently from one another; and type 3 is a near-complete deficiency (rare, most severe, usually recessively inherited). The single most important management principle is that desmopressin (DDAVP) only works in a patient who has functional VWF for it to release from endothelial Weibel-Palade bodies — it is ineffective in type 3 (there is nothing to release), is actively contraindicated in type 2B (it releases a dysfunctional VWF that binds platelets excessively, worsening thrombocytopenia), and must never be relied on for an individual patient without a documented trial-of-response confirming they actually respond.[2]
Recognize the Condition
Most patients present with a lifelong history of mucocutaneous bleeding beginning in childhood or adolescence, often with a family history, though up to about a third of confirmed cases have no clear family history at diagnosis (variable penetrance, recessive inheritance in type 3, or a de novo variant). A structured bleeding history — using the ISTH Bleeding Assessment Tool (ISTH-BAT), a standardized questionnaire and scoring system validated across inherited bleeding disorders — is the recommended way to distinguish a genuinely bleeding-disorder-consistent history from ordinary nuisance bruising or an isolated borderline lab value, since patient recall of "easy bruising" alone is notoriously unreliable and over-inclusive.[3]
- Epistaxis that is frequent, prolonged (over 10 minutes), bilateral, or has required medical attention or cautery.
- Easy or spontaneous bruising, often out of proportion to any identifiable trauma.
- Gum bleeding with tooth-brushing or minor dental work.
- Heavy menstrual bleeding since menarche — frequently the presenting complaint in adolescent girls and young women, and a strong prompt to test for VWD (see Special Populations and the Heavy Menstrual Bleeding chapter).
- Prolonged bleeding after a minor cut, tooth extraction, tonsillectomy, or other surgery.
- Postpartum hemorrhage, including delayed postpartum hemorrhage occurring after initial normal bleeding (see Special Populations).
- Less commonly, gastrointestinal bleeding, particularly with an angiodysplastic lesion in more severe or acquired VWD.
In more severe disease — type 3 in particular, and type 2N, which is often initially mistaken for mild-to-moderate hemophilia A — hemarthrosis and deep muscle bleeding can occur from the associated FVIII deficiency, a pattern that should prompt VWD-specific testing (not just a factor VIII assay) rather than an assumption of hemophilia A, since the inheritance pattern, family screening implications, and correct replacement product differ between the two.
Risk Factors
- A first-degree relative with VWD or an unexplained bleeding history — though a negative family history does not exclude VWD, particularly type 3 (recessive, both parents may be unaffected carriers) or a de novo variant.
- Consanguinity, which increases the likelihood of type 3 disease specifically.
- Blood group O — independently associated with VWF levels roughly 25-30% lower than non-O blood groups as a normal population variant, not itself pathologic, but relevant when interpreting a borderline VWF level in someone with group O blood.
- Female sex — bleeding symptoms are more often clinically apparent because menstruation and childbirth are recurring hemostatic challenges that a male with the same VWF level may never encounter.
- Concurrent use of a drug that impairs platelet function or coagulation (NSAIDs, antiplatelet agents, anticoagulants), which can unmask or significantly worsen bleeding in previously mild or undiagnosed VWD.
- An underlying condition associated with acquired von Willebrand syndrome — a distinct, later-in-life, non-inherited entity without a personal or family bleeding history, seen with lymphoproliferative or myeloproliferative disorders, marked thrombocytosis (which adsorbs VWF onto platelets), hypothyroidism, or high-shear cardiac conditions such as severe aortic stenosis or a ventricular assist device (Heyde syndrome describes the aortic-stenosis association) — distinguishing acquired from inherited VWD changes both the workup (treat the underlying condition) and the family-screening implications.
Red Flags & Refer
Escalate immediately or refer urgently for any of the following:
- Severe or life-threatening bleeding of any kind — gastrointestinal hemorrhage, intracranial hemorrhage, major trauma-related bleeding, or postpartum hemorrhage — needs emergency stabilization and immediate hemostatic treatment per the patient's own action plan (or urgent hematology consultation if none exists), not a routine outpatient visit.
- A newly suspected bleeding disorder presenting with a hemophilia-like pattern — spontaneous hemarthrosis or deep muscle bleeding — needs urgent hematology referral rather than being managed as an isolated musculoskeletal injury; this pattern should specifically prompt VWD testing (VWF:Ag, VWF activity, FVIII:C, and reflex to a VWF:FVIII binding assay) rather than assuming hemophilia A from a low FVIII:C alone, since type 2N VWD produces an identical routine-lab picture.
- Suspected type 2B VWD (thrombocytopenia that worsens with physiologic stress, pregnancy, or a prior desmopressin dose) — do not give empiric desmopressin; refer for confirmatory testing (RIPA, multimer analysis) before any hemostatic treatment decision.[2]
- Any planned surgery, invasive procedure, or dental extraction in a patient with known or suspected VWD who does not yet have a documented type and desmopressin-responder status — refer for pre-procedure hematology assessment well in advance, not on the day of the procedure.
- A pregnant patient with known VWD approaching delivery needs a peripartum hemostasis management plan arranged by hematology and obstetric anesthesia well before labour, given the expected postpartum fall in VWF/FVIII levels (see Special Populations).
- A patient with known VWD who is being started on an antiplatelet agent or anticoagulant for an unrelated indication (new atrial fibrillation, an acute coronary syndrome, venous thromboembolism) should be flagged for specialist input before or at initiation, not after a bleeding event occurs.
- Bleeding that is excessive or prolonged despite correctly dosed desmopressin or VWF concentrate is a treatment failure requiring urgent specialist reassessment — do not simply repeat the same dose (see Treatment Failure & Next Step).
Assessment & Diagnosis
History
Take a structured bleeding history using the ISTH-BAT, construct a family pedigree, and take a detailed menstrual, obstetric, surgical, and dental bleeding history alongside a full medication review (NSAIDs, antiplatelets, anticoagulants, and any supplement with antiplatelet activity).[3]
Initial laboratory panel
- CBC with platelet count and blood smear — a low or borderline-low platelet count raises the possibility of type 2B, and the smear can also reveal a coexisting platelet disorder.
- PT and aPTT — the aPTT may be prolonged if FVIII is sufficiently reduced (type 3, type 2N, or severe type 1); the PT is normal in VWD.
- VWF antigen (VWF:Ag) — the total quantity of VWF protein present.
- VWF activity — traditionally the ristocetin cofactor assay (VWF:RCo), increasingly being replaced by the VWF:GPIbM assay, which measures the same platelet-binding function with better precision and reproducibility; use whichever is available locally.
- FVIII activity (FVIII:C).
Testing caveats
VWF is an acute-phase reactant and its levels are also raised by stress, exercise, pregnancy, and estrogen exposure — any of these can transiently normalize a mild underlying deficiency and produce a false-negative result. Avoid testing during acute illness or inflammation where possible, and repeat testing is often needed before excluding a mild phenotype, particularly when the bleeding history remains clinically convincing despite a first normal result.[1]
Type classification
Interpreting the VWF:Ag, VWF activity, and FVIII:C pattern
Type 1 — partial quantitative deficiency
VWF:Ag and VWF activity are both reduced roughly proportionately (activity:antigen ratio at least 0.7), FVIII:C is proportionately reduced or near normal, and multimer distribution is normal.
Type 2 — qualitative dysfunction (activity:antigen ratio under 0.7)
Disproportionately reduced activity relative to antigen triggers reflex testing to subtype:
- RIPA (ristocetin-induced platelet aggregation) at low-dose ristocetin — markedly increased aggregation suggests type 2B (or, less commonly, the distinct platelet-receptor disorder platelet-type VWD, which mimics 2B on RIPA but needs genetic/mixing-study differentiation since its correct treatment is platelet concentrate, not VWF concentrate); a normal or decreased response suggests 2A or 2M.
- Multimer analysis — loss of high-molecular-weight multimers indicates 2A or 2B; a normal multimer distribution with reduced activity indicates 2M.
- VWF:FVIII binding assay — markedly reduced VWF-FVIII binding with an otherwise normal VWF:Ag, activity, and multimer pattern identifies type 2N, which mimics mild-to-moderate hemophilia A on routine testing (isolated low FVIII:C) — confirming 2N matters because it needs a VWF-containing concentrate rather than an FVIII-only product, and because its autosomal (not X-linked) inheritance changes family counselling.
Type 3 — near-complete deficiency
VWF:Ag and VWF activity are essentially undetectable (typically under 3-5 IU/dL), and FVIII:C is markedly reduced (often under 10 IU/dL, hemophilia-range) because there is no VWF left to stabilize it.
Genetic testing is increasingly used to confirm type 2 subtypes (particularly distinguishing 2B from platelet-type VWD) and to counsel families about type 3's recessive inheritance, but is not required to establish an initial diagnosis or treatment plan in most patients.[1]
A documented desmopressin trial-of-response (see Choose Initial Treatment) is itself part of the diagnostic-to-treatment-planning workup — every patient being considered for desmopressin-based management needs this on record before it is relied on clinically for a bleed or a procedure.[2]
Treatment Targets
- Control active bleeding and prevent bleeding matched to the specific clinical situation — spontaneous mucocutaneous bleeding, a planned minor or major procedure, or an emergency.
- Establish and document each patient's desmopressin-responder status (or contraindication) proactively, before it is urgently needed.
- Achieve the specific VWF activity and FVIII:C targets appropriate to the bleeding risk of a planned procedure (minor versus major).
- Manage heavy menstrual bleeding and peripartum bleeding risk specifically in women with VWD, coordinated with the general HMB approach.
- Avoid avoidable iatrogenic bleeding risk — screen for and avoid unnecessary NSAIDs, antiplatelet agents, and anticoagulants.
- Avoid iatrogenic harm from treatment itself — supratherapeutic FVIII levels from repeated VWF concentrate dosing, and thrombosis risk with tranexamic acid in a patient who has other prothrombotic risk factors.
- Support genetic counselling and cascade family screening given the inherited nature of the disease.
Choose Initial Treatment
Type 1, and desmopressin-responsive type 2A/2M/2N
1st line desmopressin (DDAVP), but only after a documented trial-of-response for that specific patient — measure VWF activity, VWF:Ag, and FVIII:C at baseline and again at 1 hour and 4 hours after a trial dose; response is generally defined as a rise to at least 50 IU/dL. The 1-hour level matters specifically for suspected type 1C (accelerated VWF clearance), whose levels can fall sharply by 4 hours despite an adequate 1-hour peak — without checking both timepoints, a type 1C patient can appear to respond adequately yet lose hemostatic coverage well before the next planned dose.[2]
Type 2B
Contraindicated desmopressin is generally contraindicated in type 2B — it releases the same dysfunctional VWF that already binds platelets excessively, and can precipitate or worsen thrombocytopenia. Use VWF concentrate as first-line treatment instead, without a desmopressin trial.[2]
Type 3, confirmed desmopressin non-responders, and any major surgery
1st line VWF concentrate replacement — required in type 3 (there is no endogenous VWF for desmopressin to release), in confirmed non-responders of any type, and for major surgery in every VWD patient regardless of documented desmopressin-responder status: the 2021 guideline specifically advises against relying on desmopressin for major surgery even in a documented responder, given the more sustained and predictable coverage VWF concentrate provides.[2]
Mild mucosal bleeding, or as an adjunct
Tranexamic acid alone for minor mucosal bleeding (epistaxis, gum bleeding, minor dental work) or heavy menstrual bleeding, or combined with desmopressin or VWF concentrate for added effect around a larger bleed or procedure.
Heavy menstrual bleeding
VWD-specific approach manage per the general Heavy Menstrual Bleeding chapter's workup and first-line therapy (tranexamic acid, hormonal therapy including the levonorgestrel-releasing intrauterine system) as the foundation, adding intranasal desmopressin on heavy-flow days for a documented, non-2B responder with breakthrough bleeding, or VWF concentrate for severe or refractory bleeding — see Special Populations and Clinical Decision Algorithm.
Perioperative and dental bleeding-risk management
Structured pathway see Clinical Decision Algorithm for the minor-versus-major procedure decision tree, and Drug Tables for exact dosing.
Interaction screening
Avoid without specialist input NSAIDs, antiplatelet agents, and anticoagulants in any patient with known VWD; if one is genuinely needed for an unrelated indication, involve hematology before starting rather than after a bleeding complication.
Non-Pharmacologic Treatment
- Genetic counselling and cascade testing of first-degree relatives given the inherited nature of VWD.
- Ensure every patient carries medical alert identification stating their VWD type and desmopressin-responder status, and an individualized bleeding action plan — particularly important when travelling.
- Avoid intramuscular injections where an alternative route is feasible (hematoma risk); when an IM injection genuinely cannot be avoided (for example, a required vaccine), apply firm, extended direct pressure to the injection site afterward.
- Local hemostatic measures for minor bleeding or dental work: direct pressure, ice, nasal packing for epistaxis, topical thrombin or a fibrin sealant, and an antifibrinolytic mouthwash before and after dental procedures.
- Individualize activity counselling — avoid contact sports and other high bleeding-risk activities in more severe disease, while encouraging normal activity in mild, well-controlled VWD.
- Treat iron-deficiency anemia directly with iron repletion in anyone with chronic blood loss (heavy menstrual bleeding, recurrent epistaxis) rather than assuming it will resolve once bleeding is controlled.
- Arrange the hemostatic plan for any planned surgery or dental extraction well in advance of the appointment, not on the day of the procedure.
- Hepatitis A and B vaccination is recommended for anyone who may receive plasma-derived blood products, consistent with standard comprehensive bleeding-disorder care.
Special Populations
Pregnancy
VWF and FVIII both rise physiologically through pregnancy — by up to roughly 3-fold above baseline by the third trimester — which can partly mask mild VWD on antenatal testing. These levels then fall rapidly over the first 1-2 weeks postpartum, creating a real risk of delayed postpartum hemorrhage after apparently normal delivery bleeding, particularly in type 1 with a lower baseline level and in any type 2 or type 3 disease. Neuraxial anesthesia (epidural or spinal) generally requires a VWF activity and FVIII:C of at least 50 IU/dL, individualized with obstetric anesthesia. Tranexamic acid is commonly used peripartum. Desmopressin can be used in a documented responder for minor peripartum bleeding, with fluid restriction and sodium monitoring, but is not the standard approach to hemostatic coverage for delivery itself; VWF concentrate is used when physiologic levels are not adequately elevated, for type 3, type 2B, or a non-responder, or for major bleeding. Monitor VWF activity and FVIII:C through pregnancy and for at least 2 weeks postpartum, and arrange this plan with hematology and obstetric anesthesia well before labour, not on admission.[2]
Neonates and pediatrics
Check cord blood or neonatal FVIII and VWF levels when type 3 or type 2N is suspected antenatally. Apply firm, extended pressure after any intramuscular injection (including routine vitamin K) in a newborn with suspected severe VWD. A structured bleeding history can be harder to elicit in young children with a limited bleeding-exposure history; desmopressin is generally avoided or used with particular caution under 2 years of age given a materially higher risk of hyponatremic seizures from free-water retention — strict fluid restriction is essential whenever it is used in this age group. VWF concentrate is dosed on the same weight-based (IU/kg) principle as in adults.
Renal impairment
Desmopressin's antidiuretic effect and hyponatremia risk are more pronounced with reduced renal clearance — use particular caution and closer sodium monitoring. Tranexamic acid requires dose reduction in renal impairment (see Drug Tables for the specific serum-creatinine-based adjustment).
Cardiovascular disease
Desmopressin causes a transient blood pressure change and fluid retention — use caution in cardiovascular disease or uncontrolled hypertension, and always infuse the IV dose slowly (15-30 minutes) rather than as a rapid push. Tranexamic acid is contraindicated in active thromboembolic disease or a significant thrombosis risk.
Repeated dosing / tachyphylaxis
Repeated desmopressin dosing over several days shows a diminishing response after about 3-4 doses as endothelial VWF stores are depleted — space repeated doses at least 24-48 hours apart, and switch to VWF concentrate if hemostatic coverage is needed over a longer multi-day course.[2]
Monitoring & Follow-Up
Confirm bleeding has actually stopped or that a procedure achieved adequate hemostasis clinically, not from a single reassuring lab value alone. With desmopressin, monitor serum sodium, fluid balance, and blood pressure — hyponatremia risk rises with repeated dosing, in young children, and if fluid restriction is not followed. With VWF concentrate, particularly for major surgery, monitor VWF activity and FVIII:C at the interval specified in the treatment plan (commonly every 12 hours on the day of surgery, then every 24 hours) to stay within the target range — avoid supratherapeutic FVIII levels, since FVIII can continue rising for days after VWF replacement (VWF's own stabilizing effect on endogenous and infused FVIII) and materially elevated levels carry a real thrombosis risk, particularly with a higher-VWF:FVIII-ratio product and repeated dosing.[2] For women with VWD and heavy menstrual bleeding, track bleeding pattern and quality-of-life impact and recheck CBC/ferritin as in the Heavy Menstrual Bleeding chapter. A documented type and desmopressin-responder status does not need re-testing on a routine schedule once established, but should be reconfirmed if the clinical bleeding pattern changes unexpectedly. Arrange annual comprehensive-care review at a hemophilia treatment centre or hematology clinic, as for other inherited bleeding disorders, and screen for inhibitor development — rare, seen mainly in type 3 patients with repeated VWF concentrate exposure — if response to concentrate becomes unexpectedly inadequate.
Common Errors to Avoid
- Giving desmopressin without ever having documented an actual trial-of-response for that specific patient.
- Giving desmopressin to a patient with type 2B, or with unconfirmed/unclassified VWD and thrombocytopenia.
- Relying on desmopressin instead of VWF concentrate for major surgery, even in a documented responder.[2]
- Treating type 2N as hemophilia A (an FVIII-only product) based on an isolated low FVIII:C, without confirming the diagnosis with a VWF:FVIII binding assay.
- Missing VWD in a woman with heavy menstrual bleeding since menarche because the workup stopped at a gynecologic explanation without a bleeding-disorder history and VWF panel.
- Giving an intramuscular injection without extended direct pressure afterward, or without considering an alternative route.
- Repeating desmopressin doses too close together, risking both tachyphylaxis (diminishing hemostatic response) and hyponatremia.
- Not fluid-restricting or not monitoring serum sodium after desmopressin, especially in young children.
- Missing the postpartum window when physiologically elevated VWF/FVIII levels fall after delivery, leading to a missed delayed postpartum hemorrhage risk.
- Starting an NSAID, antiplatelet agent, or anticoagulant in a known VWD patient without specialist input.
- Confusing type 2B with platelet-type VWD on a markedly increased low-dose RIPA response — the two need different products (VWF concentrate versus platelet concentrate) and are distinguished by genetic/mixing studies, not RIPA alone.
Clinical Decision Algorithm
Drug Tables
How to use this table: doses are adult doses and require patient-specific confirmation, including renal/hepatic adjustment and a documented desmopressin trial-of-response before clinical use. Select any linked drug name to open its complete Pharmasist Drug Database page.
Desmopressin (DDAVP) — for a documented responder only
Works by releasing endogenous VWF from endothelial Weibel-Palade bodies -- effective in type 1 and most desmopressin-responsive type 2A/2M/2N; ineffective in type 3 (nothing to release); contraindicated in type 2B (releases a dysfunctional VWF that worsens thrombocytopenia). Not used for major surgery even in a documented responder -- use VWF concentrate instead. Named as text only in this chapter: the Drug Database's only "desmopressin" page is the low-dose oral melt tablet dosed for diabetes insipidus/nocturnal enuresis, a different formulation, concentration, and dose entirely from the hemostatic products below.
| Drug | Dosage | Adverse effects | Drug interactions | Comments |
|---|---|---|---|---|
| Desmopressin acetate, IV/SC injection | Standard dose: 0.3 mcg/kg IV, diluted in 50-100 mL normal saline, infused over 15-30 minutes, or the same dose SC undiluted Response assessment: Repeat VWF activity, VWF:Ag, and FVIII:C at 1 hour and 4 hours post-dose; adequate response is generally a rise to at least 50 IU/dL at both timepoints Fluid management: Restrict fluids to roughly maintenance-to-1.5x maintenance and avoid hypotonic IV fluids for 24 hours after dosing | Flushing, headache, transient hypotension or tachycardia, water retention/hyponatremia (especially with repeated dosing or excess free-water intake) | Additive hyponatremia risk with other antidiuretic drugs or excess hypotonic IV fluids | Requires a documented trial-of-response (VWF activity, VWF:Ag, FVIII:C at baseline, 1h, and 4h) before relying on it clinically. Contraindicated in type 2B. Infuse IV doses slowly (15-30 minutes), never as a rapid push. Space repeated doses at least 24-48 hours apart -- tachyphylaxis develops after about 3-4 doses. |
| Desmopressin acetate, concentrated intranasal spray (1.5 mg/mL) | Body weight under 50 kg: 150 mcg -- 1 spray in one nostril Body weight 50 kg or more: 300 mcg -- 1 spray (150 mcg) in each nostril | Nasal congestion/irritation, headache, facial flushing, hyponatremia with repeated dosing | Additive hyponatremia risk with other antidiuretic drugs or excess hypotonic IV fluids | A distinct, more concentrated product from the low-dose intranasal or oral desmopressin used for diabetes insipidus or enuresis -- do not substitute those weaker products. Useful for minor mucosal bleeding, dental procedures, and breakthrough heavy-flow days in a documented, non-2B responder. |
Concentrated hemostatic desmopressin dosing above is cross-checked against the current Health Canada product monograph.[4]
Von Willebrand factor / factor VIII replacement concentrates
Required for type 3, type 2B, confirmed desmopressin non-responders, and every major surgery regardless of desmopressin-responder status. Dosed by VWF activity (VWF:RCo or VWF:GPIbM) in international units per kg -- choice between products is generally based on institutional protocol and availability rather than a clinically meaningful efficacy difference.
| Drug | Dosage | Adverse effects | Drug interactions | Comments |
|---|---|---|---|---|
| Antihemophilic factor / von Willebrand factor complex (human) | Significant bleeding or major surgery, loading dose: 40-60 IU VWF activity/kg IV (up to 60-80 IU/kg for major surgery per some protocols) Minor bleeding or minor procedure: 30-50 IU VWF activity/kg IV Maintenance: Redosed to the target level and bleeding severity, guided by repeat VWF activity/FVIII:C levels | Infusion reactions, headache, rare thrombosis (particularly with repeated dosing as FVIII accumulates), theoretical pathogen-transmission risk (plasma-derived, pathogen-reduced) | Avoid combining with other prothrombotic products without specialist direction | A higher-VWF:FVIII-ratio product -- because VWF stabilizes both endogenous and infused FVIII, FVIII levels can continue rising for 1-2 days after dosing even without a repeat dose; monitor levels closely with repeated dosing to avoid a supratherapeutic FVIII level and its associated thrombosis risk. |
| Human von Willebrand factor / coagulation factor VIII complex | Significant bleeding or major surgery, loading dose: 40-60 IU VWF activity/kg IV (up to 60-80 IU/kg for major surgery per some protocols) Minor bleeding or minor procedure: 30-50 IU VWF activity/kg IV Maintenance: Redosed to the target level and bleeding severity, guided by repeat VWF activity/FVIII:C levels | Infusion reactions, headache, rare thrombosis, theoretical pathogen-transmission risk (plasma-derived, pathogen-reduced) | Avoid combining with other prothrombotic products without specialist direction | A more balanced (approximately 1:1) VWF:FVIII ratio product -- still monitor FVIII levels with repeated dosing per protocol, though accumulation is generally less pronounced than with a higher-ratio product. |
Dosing for both plasma-derived VWF/FVIII concentrates above is cross-checked against the current ASH/ISTH/NHF/WFH international guideline and their current Health Canada product monographs.[2][5][6]
Antifibrinolytic
Effective as monotherapy for minor mucosal bleeding, and as an adjunct to desmopressin or VWF concentrate around a larger bleed or procedure, including dental extractions.
| Drug | Dosage | Adverse effects | Drug interactions | Comments |
|---|---|---|---|---|
| Tranexamic acid | Adults, standard dosing: 25 mg/kg (typically about 1.3 g) PO every 8 hours, or 10 mg/kg IV every 8-12 hours Renal impairment, SCr 120-250 micromol/L: 15 mg/kg PO or 10 mg/kg IV twice daily Renal impairment, SCr 250-500 micromol/L: 15 mg/kg PO or 10 mg/kg IV once every 24 hours Renal impairment, SCr 500 micromol/L or more: Same dose once every 48 hours | Nausea, GI upset; rare visual disturbance with prolonged use | Contraindicated with a history/risk of thrombosis or active thromboembolic disease; use caution combined with a combined hormonal contraceptive given additive VTE risk | For dental procedures, start 1-2 hours before and continue for 5-7 days; an antifibrinolytic mouthwash is also used as a local adjunct. |
Evidence note: drug inventory, dosing, adverse effects, and comments reflect standard practice cross-checked against the current ASH/ISTH/NHF/WFH international guideline and current Health Canada product monographs.[2][7]
References
- ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease — Blood Advances, American Society of Hematology (James PD, Connell NT, Ameer B, et al.). Blood Adv 2021;5(1):280-300
- ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease — Blood Advances, American Society of Hematology (Connell NT, Flood VH, Brignardello-Petersen R, et al.). Blood Adv 2021;5(1):301-325
- ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders — Journal of Thrombosis and Haemostasis, ISTH/SSC Joint VWF and Perinatal/Pediatric Haemostasis Subcommittees (Rodeghiero F, Tosetto A, Abshire T, et al.). J Thromb Haemost 2010;8(9):2063-2065
- OCTOSTIM (desmopressin acetate) Injection and Nasal Spray Product Monograph — Ferring Inc., Health Canada Drug Product Database. Health Canada Product Monograph
- HUMATE-P (antihemophilic factor / von Willebrand factor complex, human) Product Monograph — CSL Behring Canada Inc., Health Canada Drug Product Database. Health Canada Product Monograph
- WILATE (human von Willebrand factor / coagulation factor VIII complex) Product Monograph — Octapharma Canada Inc., Health Canada Drug Product Database. Health Canada Product Monograph
- CYKLOKAPRON (tranexamic acid) Product Monograph — Pfizer Canada ULC, Health Canada Drug Product Database. Health Canada Product Monograph
Professional reference only. Apply clinical judgment and verify patient-specific decisions against current authoritative guidance and product information.