Von Willebrand Disease (Bleeding)MANAGEMENT

HEMATOLOGIC AND ONCOLOGIC PRESENTATIONS · 12 SECTIONS

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377 items

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Cardiovascular Presentations71▾

Respiratory Presentations52▾

Neurological Presentations83▾

Gastrointestinal Presentations74▾

Genitourinary and Reproductive Presentations48▾

Infectious Disease Presentations148▾

Trauma Presentations89▾

Toxicologic Presentations80▾

Psychiatric Presentations26▾

Musculoskeletal and Rheumatologic Presentations96▾

Dermatology Presentations23▾

Environmental and Exposure Presentations27▾

Hematologic and Oncologic Presentations36▾

Acute chest syndrome Acute Hemolytic Transfusion Reaction Anticoagulant reversal (warfarin, DOAC, heparin)Aplastic Anemia (Crisis)Autoimmune Hemolytic Anemia CAR-T and cytokine release syndrome Coagulopathy Delayed Hemolytic Transfusion Reaction Disseminated Intravascular Coagulation (DIC)Essential Thrombocythemia Febrile Non-hemolytic Transfusion Reaction Graft Versus Host Disease (Acute)Hemolytic Uremic Syndrome (HUS)Hemophilia A (Bleeding Crisis)Heparin-Induced Thrombocytopenia (HIT)Hyperviscosity syndrome Immune checkpoint inhibitor toxicity Immune Thrombocytopenic Purpura (ITP)Leukemoid Reaction Leukostasis Massive transfusion protocol Myelofibrosis Neutropenic enterocolitis (typhlitis)Paroxysmal Nocturnal Hemoglobinuria Polycythemia Vera (Crisis)Pure Red Cell Aplasia Sickle Cell (Acute Chest Syndrome)Sickle Cell (Splenic Sequestration)Sickle cell crisis (vaso-occlusive)Symptomatic anemia Thrombocytopenia (severe)Thrombotic Thrombocytopenic Purpura (TTP)Transfusion-Associated Circulatory Overload (TACO)Transfusion-Related Acute Lung Injury (TRALI)Tumor lysis syndrome Von Willebrand Disease (Bleeding)

Pediatric-Specific Presentations19▾

Endocrine and Metabolic Presentations45▾

ENT and Maxillofacial Presentations27▾

Ophthalmologic Presentations19▾

Obstetric Presentations23▾

Systemic and Miscellaneous Presentations17▾

01Approach to the Critical Patient/attcp24

Immediate Stabilization

›Hemodynamic assessment and resuscitation priorities

›Airway, breathing, circulation in hemodynamically unstable patients

›Two large-bore IV access; fluid resuscitation for hemorrhagic shock

›Packed red blood cell transfusion for hemoglobin <70 g/L or hemodynamic compromise

›Emergent VWF/FVIII concentrate replacement triggers

›Active intracranial hemorrhage: target VWF:RCo and FVIII activity >100 IU/dL immediately

›Life-threatening GI hemorrhage with hemodynamic instability

›Uncontrolled surgical or traumatic hemorrhage in known VWD patient

›Rapidly expanding hematoma or compartment syndrome risk

Resuscitation Targets

›Hemodynamic targets during active hemorrhage

›Mean arterial pressure >65 mmHg

›Heart rate <100 beats/min

›Urine output >0.5 mL/kg/hour

Consult Triggers

›Immediate hematology consultation indications

›Unknown VWD subtype with significant active bleeding

›Type 2B or type 3 VWD with major hemorrhage

›Suspected inhibitor development (inadequate response to factor replacement)

›Need for perioperative hemostatic planning

Pitfalls

›Critical management pitfalls

›Desmopressin contraindicated in type 2B VWD (worsens thrombocytopenia)

›Desmopressin ineffective and contraindicated in type 3 VWD (no stored VWF)

›Tranexamic acid relatively contraindicated with gross hematuria (ureteral clot risk)

›Aspirin and NSAIDs must be avoided; acetaminophen preferred for analgesia

›Repeated desmopressin dosing causes tachyphylaxis within 24-48 hours

02History/hx37

Bleeding Pattern

›Mucocutaneous bleeding predominance

›Epistaxis (most common; frequency, duration, bilateral vs. unilateral)

›Easy bruising disproportionate to trauma

›Gingival bleeding with tooth brushing or spontaneous

›Prolonged bleeding from minor cuts

Menstrual and Obstetric History

›Heavy menstrual bleeding features

›Duration greater than 7 days per cycle

›Pad or tampon saturation rate greater than 1 per hour

›Clot passage greater than 1 cm diameter

›Postpartum hemorrhage history (reported in 6-59% of affected women)

Surgical and Procedural History

›Hemostatic complications with prior procedures

›Dental extraction bleeding (60-80% of VWD patients bleed excessively after dental extraction or surgery)

›Tonsillectomy or adenoidectomy complications

›Prior desmopressin challenge results (responder vs. non-responder status)

›Prior VWF/FVIII concentrate use and response

Alarm Symptoms

›High-acuity bleeding presentations

›Melena, hematochezia, or hematemesis (GI angiodysplasia, especially elderly type 2/3)

›Severe headache or altered mental status (intracranial hemorrhage, 0-8% lifetime prevalence)

›Joint swelling or pain (hemarthrosis suggests severe disease: type 3 or type 2N)

›Lightheadedness or syncope (hemorrhagic anemia)

Risk Factors

›Patient-specific bleeding risk factors

›Known VWD subtype and baseline VWF/FVIII levels

›Blood group O (associated with approximately 25% lower baseline VWF levels)

03Physical Exam/exam27

Vital Signs

›Hemodynamic stability assessment

›Tachycardia (resting heart rate >100 beats/min suggests significant blood loss)

›Hypotension (systolic BP <90 mmHg suggests hemorrhagic shock)

›Orthostatic blood pressure change (>20 mmHg systolic drop)

Skin

›Cutaneous bleeding findings

›Ecchymoses (often large and disproportionate to reported trauma)

›Petechiae (less common than in platelet disorders; suggests thrombocytopenia if prominent)

›Hematomas over pressure points or minor trauma sites

Head, Eyes, Ears, Nose, Throat

›Mucosal bleeding sites

›Active epistaxis (site, unilateral vs. bilateral, duration)

›Gingival oozing or oropharyngeal hemorrhage

›Conjunctival pallor (anemia from chronic blood loss)

Abdomen

›Abdominal hemorrhage signs

›Tenderness or guarding (GI hemorrhage or retroperitoneal bleed)

›Distension (intraperitoneal hemorrhage)

›Flank ecchymosis (Grey Turner sign: retroperitoneal hemorrhage)

Musculoskeletal

›Joint and soft tissue hemorrhage

›Joint effusion (hemarthrosis: most common in ankles, knees; suggests severe disease)

›Limited range of motion (chronic hemarthrosis sequelae)

›Calf tenderness or compartment firmness

Neurological

›CNS hemorrhage assessment

›Mental status changes or confusion

04Differential Diagnosis/ddx17

Inherited Bleeding Disorders

›Inherited coagulopathy mimics

›Hemophilia A (low FVIII, normal VWF; X-linked; deep tissue and joint bleeding predominates)

›Type 2N VWD mimics hemophilia A (impaired VWF-FVIII binding; autosomal recessive)

›Hemophilia B (low factor IX; X-linked; similar to hemophilia A clinically)

›Platelet function disorders (Glanzmann thrombasthenia, Bernard-Soulier syndrome; mucocutaneous bleeding similar to VWD; distinguished by platelet aggregation studies)

Acquired Coagulopathy

›Acquired bleeding disorders

›Acquired von Willebrand syndrome (lymphoproliferative disorders, aortic stenosis, hypothyroidism, autoimmune disease; new-onset bleeding in older adults without family history)

›Liver disease with synthetic dysfunction (impaired coagulation factor production; check PT/INR)

›Disseminated intravascular coagulation (consumption of coagulation factors; microangiopathic hemolytic anemia)

Thrombocytopenic Disorders

›Platelet count disorders

›Immune thrombocytopenic purpura (isolated thrombocytopenia; normal coagulation studies)

›Thrombotic thrombocytopenic purpura (thrombocytopenia, microangiopathic hemolytic anemia, neurological signs)

›Heparin-induced thrombocytopenia (thrombocytopenia 5-14 days after heparin exposure)

Other Considerations

›Additional differential diagnoses

›Drug-induced platelet dysfunction (aspirin, clopidogrel, GPIIb/IIIa inhibitors)

›Child abuse (pediatric patients with unexplained bruising; VWD workup helps exclude)

›Ehlers-Danlos syndrome (connective tissue disorder with easy bruising; normal coagulation)

05Laboratory Tests/lt24

First-Line Coagulation Panel

›Initial diagnostic laboratory studies

›Complete blood count with platelet count (thrombocytopenia in type 2B)

›PT/INR (usually normal in VWD; prolonged in liver disease or DIC)

›aPTT (may be prolonged if FVIII is significantly reduced, particularly in types 2N and 3)

VWF-Specific Testing

›Confirmatory VWD laboratory panel

›VWF antigen (VWF:Ag): quantifies total VWF protein concentration

›Definite VWD: VWF:Ag less than or equal to 30 IU/dL

›Low VWF or possible VWD: 30-50 IU/dL

›VWF activity (VWF:RCo, VWF:GPIbR, or VWF:GPIbM): measures platelet-binding function

›Factor VIII activity (often reduced in types 2N and 3; may be very low in type 3)

Subtyping Tests

›Second-line VWD classification studies

›VWF activity to VWF:Ag ratio

›Ratio less than or equal to 0.6 suggests type 2 qualitative defect

›VWF multimer analysis (distinguishes subtypes 2A, 2B, 2M; absent large multimers in 2A)

›Low-dose ristocetin-induced platelet aggregation (RIPA): enhanced aggregation in type 2B

›VWF:FVIII binding assay (abnormal in type 2N)

›VWF propeptide assay (helps distinguish type 1 from type 3; elevated ratio indicates increased clearance)

Adjunct Laboratory Studies

›Supporting laboratory investigations

›Iron studies and ferritin (chronic blood loss from menorrhagia or GI bleeding)

›Type and screen (anticipate transfusion in moderate/severe bleeding)

›ABO blood group (blood group O associated with approximately 25% lower VWF levels)

›Thyroid function tests (hypothyroidism lowers VWF levels; particularly in acquired presentations)

›Peripheral blood smear (schistocytes in TTP/DIC; lymphoma cells in acquired vWS)

›Hepatic function panel (synthetic function assessment for coagulopathy)

06Diagnostic Tests/dt20

Scoring Systems

›ISTH Bleeding Assessment Tool (BAT)

›Standardized scoring system for bleeding symptom severity quantification

›Useful for screening and diagnosis of bleeding disorders in clinic settings

›Limitations include cumulative scoring and age-dependent thresholds

›Abnormal BAT score increases probability of VWD diagnosis in symptomatic patients

MRI

›MRI indications in VWD

›Joint MRI for hemarthrosis characterization and arthropathy staging in severe disease

›Brain MRI for subacute intracranial hemorrhage or venous sinus thrombosis evaluation

›MRI superior to plain radiographs for early synovial and cartilaginous joint damage detection

›MRI abdomen/pelvis for retroperitoneal hematoma delineation when CT contrast contraindicated

›CT indications by hemorrhage site

›CT head without contrast for acute intracranial hemorrhage (indication: headache, altered mental status, focal neurological deficits)

›CT abdomen and pelvis with contrast for suspected intra-abdominal or retroperitoneal hemorrhage

›CT angiography for localizing active arterial GI hemorrhage

›Not indicated for VWD diagnosis itself; used for complication assessment

Ultrasound

›Point-of-care and formal ultrasound applications

›FAST exam for intraperitoneal free fluid in hemodynamically unstable patients

›Joint ultrasound for hemarthrosis detection and aspiration guidance

›Soft tissue ultrasound for hematoma assessment and compartment syndrome evaluation

›Transthoracic echocardiography in acquired VWS to evaluate for aortic stenosis (jet velocity greater than 4 m/s or valve area less than 1.0 cm2 associated with high-shear VWF proteolysis)

07Disposition/dispo21

Admission Indications

›Criteria for inpatient admission

›Hemodynamically significant active hemorrhage requiring IV factor replacement

›Intracranial hemorrhage (ICU-level care required)

›GI hemorrhage (hemodynamically significant or requiring endoscopic intervention)

›Postpartum hemorrhage with ongoing blood loss

›Severe anemia (hemoglobin <70 g/L) requiring transfusion

›Need for serial VWF/FVIII activity monitoring and repeated dosing

Observation Indications

›Criteria for ED observation or short-stay admission

›Moderate bleeding controlled with desmopressin requiring monitoring for rebleeding within 8-12 hours

›Post-desmopressin hyponatremia monitoring (particularly pediatric patients and those with fluid restriction non-compliance)

›Moderate anemia requiring monitoring without immediate transfusion trigger

Discharge Criteria

›Safe discharge criteria

›Minor mucosal bleeding controlled with local measures and oral tranexamic acid

›Known mild type 1 VWD with established outpatient treatment plan

›Hemodynamically stable with no active ongoing bleeding

›Reliable follow-up arranged with hematology within 1-2 weeks

Consultations

›Subspecialty consultation indications

›Hematology: unknown subtype, type 2B or type 3, factor replacement need, perioperative planning

›Gastroenterology: GI hemorrhage or known/suspected angiodysplasia

›Gynecology: heavy menstrual bleeding or obstetric hemorrhage

›Interventional radiology: angioembolization for uncontrolled visceral hemorrhage

08Treatment/t74

Minor Mucosal Bleeding

›First-line management for epistaxis, oral, and minor wound bleeding

›Local hemostatic measures

›Nasal packing with thrombin-soaked pledgets or Merocel for epistaxis

›Direct sustained pressure for at least 10-15 minutes for wound bleeding

›Topical tranexamic acid solution 4.8% applied directly to mucosa

›Tranexamic acid systemic dosing

›1 g PO three times daily for 5-7 days for mucosal bleeding

›1 g IV over 10 minutes for acute severe mucosal bleeding

›Relatively contraindicated with gross hematuria (ureteral clot obstruction risk)

Desmopressin (DDAVP)

›Desmopressin dosing and indications

›IV formulation

›0.3 mcg/kg IV in 50 mL normal saline over 15-30 minutes

›Raises VWF and FVIII 2-4 fold within 30-60 minutes; duration 8-12 hours

›Maximum dose 20 mcg; limit repeat dosing to 24-48 hours due to tachyphylaxis

›Intranasal formulation (Stimate)

›Weight less than 50 kg: 150 mcg (one spray per nostril not recommended as single spray)

›Weight 50 kg or greater: 300 mcg (one spray per nostril)

›Subcutaneous formulation

›0.3 mcg/kg SC for patients without IV access

›Desmopressin indications

›Type 1 VWD with confirmed prior desmopressin challenge response

›Minor to moderate bleeding in known responders

›Desmopressin contraindications

›Type 2B VWD (worsens thrombocytopenia by releasing abnormal VWF)

›Type 3 VWD (no stored VWF; ineffective)

›Generally ineffective in types 2A, 2M, 2N

›Children under 2 years (high risk of hyponatremic seizures)

›Post-desmopressin monitoring

›Fluid restriction to 1 L per 24 hours for 24 hours post-dose

›Serum sodium at 4-8 hours post-dose in children and elderly (10% incidence hyponatremia)

VWF/FVIII Concentrate Replacement

›Factor concentrate indications and dosing

›Indications for concentrate use

›Type 2 and type 3 VWD with significant bleeding

›Desmopressin non-responders or unknown responder status with active hemorrhage

›Major surgery, trauma, or life-threatening hemorrhage in any VWD type

›Plasma-derived VWF/FVIII concentrates (Humate-P, Wilate)

›Loading dose: 40-80 VWF:RCo IU/kg IV for life-threatening hemorrhage

›Target VWF:RCo and FVIII activity greater than 100 IU/dL for major/life-threatening bleeding

›Target VWF:RCo greater than 50 IU/dL for minor bleeding or surgery

›Redosing every 8-12 hours to maintain hemostatic levels

›Recombinant VWF (Vonvendi)

›Approved for patients 18 years and older

›Loading dose: 40-80 IU/kg VWF:RCo IV; add FVIII concentrate if FVIII levels critically low

›Does not contain FVIII; supplement with FVIII concentrate if FVIII activity very low

›Type 2N and type 3 VWD product preference

›Prefer products containing both VWF and FVIII (e.g., Humate-P) to restore both deficiencies

›Monitoring during factor replacement

›VWF:RCo and FVIII activity levels at 1 hour post-infusion and every 12 hours during therapy

Blood Product Support

›Adjunctive transfusion therapy

›Cryoprecipitate (only if VWF/FVIII concentrates unavailable)

›Each unit contains approximately 80-100 IU VWF and 80-100 IU FVIII

›Dose: 1 unit per 10 kg body weight IV; less preferred due to variable content and infection risk

›Fresh frozen plasma (last resort only; multiple units required for therapeutic VWF levels)

›Packed red blood cells for hemoglobin less than 70 g/L or hemodynamic compromise

›Platelet transfusion for type 2B VWD with severe thrombocytopenia and active bleeding

Hormonal Therapy

›Hormonal management for heavy menstrual bleeding

›Combined oral contraceptives (first-line per ASH/ISTH/NHF/WFH guidelines for HMB in VWD)

›Increase VWF levels 2-3 fold via estrogen-mediated VWF synthesis upregulation

›Levonorgestrel-releasing intrauterine device (52 mg)

›Reduces menstrual blood loss by 80-90% within 3-6 months

›Preferred over desmopressin for long-term HMB management per ASH/ISTH/NHF/WFH guidelines

›Norethindrone acetate 5-15 mg PO daily for acute heavy menstrual bleeding crisis

Prophylaxis

›Long-term prophylaxis indications

›Type 3 VWD or severe type 2 VWD with recurrent disabling or life-threatening bleeding

›VWF/FVIII concentrate prophylaxis: 20-40 IU/kg two to three times weekly

›European Journal of Haematology 2022 cohort study supports prophylaxis efficacy in reducing bleeding episodes

›Iron supplementation for chronic blood loss (target ferritin greater than 30 mcg/L)

Medications to Avoid

›Hemostasis-impairing agents

›Aspirin and other cyclooxygenase inhibitors (impair platelet thromboxane synthesis)

›Clopidogrel and other P2Y12 inhibitors

›NSAIDs (brief use only if essential; acetaminophen preferred)

›Fish oil supplements (greater than 3 g/day impairs platelet aggregation)

›Vitamin E in high doses (impairs platelet function)

›Turmeric/curcumin, ginkgo biloba (antiplatelet effects)

09Special Populations/sp49

Pregnancy

›VWD management in pregnancy and the peripartum period

›Physiologic changes in pregnancy

›VWF levels rise during pregnancy (especially in third trimester); type 1 patients may have near-normal levels at term

›VWF levels may NOT rise in type 3 and some type 2 subtypes; do NOT assume hemostatic cover without testing

›Postpartum drop in VWF levels creates highest-risk period for hemorrhage (days 1-5 postpartum)

›Antepartum management

›VWF:RCo and FVIII activity at 28-34 weeks gestation to guide delivery planning

›Hematology consultation recommended for all VWD patients in pregnancy

›Target VWF:RCo and FVIII greater than 50 IU/dL for vaginal delivery; greater than 100 IU/dL for cesarean section

›Postpartum hemorrhage

›Reported in 6-59% of women with VWD (vs. 1-5% in general population)

›Tranexamic acid 1 g IV at delivery as prophylaxis (Class I evidence for postpartum hemorrhage)

›VWF/FVIII concentrate if VWF levels insufficient at delivery

›Desmopressin use with caution postpartum (fluid restriction feasibility limited by breastfeeding)

›Neuraxial anesthesia considerations

›Epidural and spinal anesthesia generally safe if VWF:RCo and FVIII greater than 50 IU/dL

›Anesthesia team must be informed of VWD subtype and current hemostatic levels

Geriatric

›VWD considerations in elderly patients

›Acquired von Willebrand syndrome prevalence

›New-onset bleeding in older adults without family history should prompt evaluation for lymphoproliferative disorder, aortic stenosis, or hypothyroidism

›Aortic stenosis (valve area less than 1.0 cm2) causes high-shear proteolysis of large VWF multimers

›GI angiodysplasia

›Most common life-threatening complication in elderly type 2 and type 3 patients

›Heyde syndrome: aortic stenosis combined with GI angiodysplasia and acquired VWS

›Upper and lower endoscopy for active GI hemorrhage evaluation

›Desmopressin cautions in elderly

10Background/b40

Epidemiology

›VWD prevalence and distribution

›VWD trait prevalence approximately 1% of the general population

›Symptomatic disease affecting approximately 0.1% of the population

›Type 1 VWD: 60-80% of all VWD cases; partial quantitative deficiency

›Type 2 VWD: approximately 20% of cases; qualitative defects in VWF function

›Type 3 VWD: less than 5% of cases; near-complete VWF absence; most severe phenotype

›Female sex increases clinical detection due to menorrhagia and obstetric hemorrhage presentations

›Postpartum hemorrhage rate in VWD: 6-59% versus 1-5% in the general population

›Intracranial hemorrhage lifetime prevalence: 0-8% in severe VWD

Pathophysiology

›Mechanisms of VWD subtypes

›Normal VWF function

›VWF is a glycoprotein secreted by endothelial cells and megakaryocytes

›Mediates platelet adhesion to subendothelial collagen at sites of vascular injury

›Protects factor VIII from premature proteolysis; acts as a chaperone protein

›Released from Weibel-Palade bodies upon endothelial activation (including by desmopressin)

›Type 1 VWD pathophysiology

›Partial quantitative reduction of structurally normal VWF

›Multiple molecular mechanisms including reduced synthesis, increased clearance, or Weibel-Palade body trafficking defects

›Type 2 VWD subtypes and mechanisms

›Type 2A: loss of high-molecular-weight VWF multimers; impaired platelet binding

›Type 2B: gain-of-function mutation; enhanced platelet binding causes spontaneous VWF-platelet aggregation; depletes high-molecular-weight multimers; causes thrombocytopenia

›Type 2M: reduced platelet-dependent VWF function without multimer loss

›Type 2N: impaired VWF-FVIII binding; mimics hemophilia A with low FVIII but normal VWF:Ag

›Type 3 VWD pathophysiology

›Near-complete or complete absence of VWF (homozygous or compound heterozygous mutations)

›Severe mucocutaneous and joint bleeding; FVIII critically low (secondary to VWF deficiency)

11Patient Discharge Instructions/pdi27

copy discharge instructions

›Von Willebrand Disease discharge instructions

›Condition overview

›Your bleeding disorder means your blood does not clot normally

›You may bleed longer than usual from cuts, dental work, or minor injuries

›Carry your medical alert identification at all times

›Medications

›Take tranexamic acid as prescribed to reduce mucosal bleeding

›Do NOT take aspirin, ibuprofen, naproxen, or other NSAIDs; use acetaminophen for pain instead

›Do not start any new medications or supplements (including fish oil, vitamin E, turmeric) without discussing with your hematologist

›Activity restrictions

›Avoid contact sports and high-risk activities that increase injury risk

›Use protective equipment (helmets, pads) for any physical activity

›No invasive procedures (dental work, piercings, injections) without prior hematology clearance

›Return to emergency department immediately for

›Bleeding that does not stop after 15-20 minutes of direct firm pressure

›Nosebleed that does not stop after 30 minutes or causes difficulty breathing

›Blood in vomit, stool (black or red), or urine

›Severe headache, sudden vision changes, confusion, weakness, or numbness

›Swollen, painful joint after minor injury (possible hemarthrosis)

›Lightheadedness, fainting, or rapid heart rate

›Heavy menstrual bleeding soaking more than one pad per hour for two or more consecutive hours

›Signs of severe anemia: extreme fatigue, shortness of breath at rest, chest pain

›Follow-up

›Hematology appointment within 1-2 weeks of this visit

›Annual comprehensive review at a bleeding disorders center (CBC, iron studies, VWF levels)

›Ensure hepatitis B vaccination is up to date

›Carry your treatment plan and factor products when traveling

12References/r17

Guidelines and Key Sources

›Primary clinical guidelines and landmark references

›Seidizadeh O, Eikenboom JCJ, Denis CV, et al. Von Willebrand Disease. Nature Reviews Disease Primers. 2024. PMID 39054329

›Leebeek FW, Eikenboom JC. Von Willebrand Disease. New England Journal of Medicine. 2016. https://www.nejm.org/doi/full/10.1056/NEJMra1601561

›Castaman G, Katsarou O, Jansen N, et al. Clinical, economic, and health-related quality of life burden associated with von Willebrand disease in adults and children. Haemophilia. 2023. https://onlinelibrary.wiley.com/doi/10.1111/hae.14655

›Rugeri L, Harroche A, Repessee Y, et al. Effectiveness of long-term prophylaxis using pdFVIII/VWF concentrate in patients with inherited von Willebrand disease. European Journal of Haematology. 2022. https://onlinelibrary.wiley.com/doi/10.1111/ejh.13778

›Jones DE, Walker JJ, Abellada AMP. Hematologic Emergencies: Recognition and Initial Management. American Family Physician. 2024. PMID 39028783

›Srivaths L, Kouides PA. Low von Willebrand Factor in Children and Adolescents: A Review. JAMA Pediatrics. 2021. https://jamanetwork.com/journals/jamapediatrics/fullarticle/10.1001/jamapediatrics.2021.2186

›Castaman G. How I Treat Von Willebrand Disease. Thrombosis Research. 2020. PMID 32819724

›Mohinani A, Patel S, Tan V, et al. Desmopressin as a hemostatic and blood sparing agent in bleeding disorders. European Journal of Haematology. 2023. https://onlinelibrary.wiley.com/doi/10.1111/ejh.13930

›Johnsen J. Von Willebrand Disease. GeneReviews [Internet]. 2024. https://www.ncbi.nlm.nih.gov/books/n/gene/von-willebrand/

›Tosetto A, Castaman G. How I Treat Type 2 Variant Forms of Von Willebrand Disease. Blood. 2015. PMID 25477497

›Sharma R, Haberichter SL. New Advances in the Diagnosis of Von Willebrand Disease. Hematology American Society of Hematology Education Program. 2019. PMID 31808831

›Yawn B, Nichols WL, Rick ME. Diagnosis and Management of Von Willebrand Disease: Guidelines for Primary Care. American Family Physician. 2009. PMID 19961139

Additional Sources

›Supporting references

›Castaman G, Borchiellini A, Coppola A, et al. Use of the von Willebrand factor concentrate with low factor VIII content in patients with inherited von Willebrand disease. European Journal of Haematology. 2022. https://onlinelibrary.wiley.com/doi/10.1111/ejh.13785

›Rare bleeding disorders: Advances in management. Haemophilia. 2024. https://onlinelibrary.wiley.com/doi/10.1111/hae.14986

›Gary M. Woods, Robert F. Sidonio. Coagulation concentrates for inherited bleeding disorders. Rossi Principles of Transfusion Medicine 6e. 2022. https://onlinelibrary.wiley.com/doi/10.1002/9781119719809.ch37

Learn More: Von Willebrand Disease (Bleeding)→

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Reviewed by SymptomDx Medical Team · Last reviewed August 18, 2026

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