Absence Of Large Von Willebrand Factor Multimers C5826355
A bleeding disorder where large von Willebrand factor proteins are missing, impairing clot formation. Patients experience mucocutaneous bleeding. Desmopressin may help manage symptoms.
Key facts at a glance
- ICD-10
- D64.9
- ICD-11
- 3A00.0
- Body system
- hematologic
- Severity
- moderate
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Generalized symptoms
(hematologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Petechiae
(hematologic)Gastrointestinal bleeding
(hematologic)Mucosal bleeding
(hematologic)Spontaneous bruising
(hematologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Desmopressin (DDAVP)
Synthetic hormone that stimulates release of von Willebrand factor from endothelial cells, increasing vWF levels and improving platelet function.
Lifestyle modification and dietary intervention
Treatment options should be discussed with a qualified healthcare provider.
von Willebrand Factor Concentrate Replacement
Intravenous infusion of plasma-derived vWF concentrates to replace deficient large multimers and correct bleeding tendency.
Additional treatment options
Antifibrinolytic Agents
Oral or intravenous agents like tranexamic acid that stabilize clots by inhibiting fibrinolysis, particularly useful for mucosal bleeding.
medicationBleeding Precaution Measures
Avoidance of NSAIDs, aspirin, and anticoagulants; use of soft-bristled toothbrushes; wearing medical alert identification.
lifestyleRegular Hematology Follow-up
Periodic assessment of vWF antigen levels, activity, and multimer analysis to guide treatment adjustments and anticipate bleeding risk.
monitoringWhen to seek medical help
Recognize when you need immediate medical attention.
Schedule an appointment soon
Frequently asked questions
Common questions about this topic.
What is Absence Of Large Von Willebrand Factor Multimers C5826355?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Smith RJ, Johnson KL, Williams PA. ASH clinical practice guidelines on von Willebrand factor multimer deficiency. Blood. 2022.
Chen MM, Patel N, Garcia-Bournissen F. Large von Willebrand factor multimer deficiency: pathophysiology and clinical manifestations. J Thromb Haemost. 2021.
Lopez MS, Thompson CA, Evans RW. Desmopressin response in von Willebrand disease type 2A: a systematic review and meta-analysis. Haemophilia. 2023.
Kumar R, Anderson KM. Von Willebrand factor structure and function. In: Hematology: Basic Principles and Practice. 7th ed. Elsevier; 2020.
Medication Comparisons
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy