Abnormal Presence Of Ultra Large Von Willebrand Factor Multimers C4022615
Ultra large von Willebrand factor multimers are abnormally large versions of a blood clotting protein that do not function properly. This is typically associated with von Willebrand disease, a genetic bleeding disorder. Treatment focuses on managing bleeding symptoms and may include clotting factor replacements.
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.
Warning signs - seek immediate care
Acute kidney injury
Neurological deficits (confusion, focal weakness)
Generalized symptoms
(hematologic)Noticeable symptoms that may vary in intensity over time.
Frequency: Common at all stages
Thrombocytopenia
(hematologic)Easy 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
Behavioral and nutritional therapy
Treatment options should be discussed with a qualified healthcare provider.
Plasma Exchange Therapy
Therapeutic plasma exchange to remove ultra-large von Willebrand factor multimers and restore normal vWF function in conditions like thrombotic thrombocytopenic purpura (TTP). Daily exchanges are typically performed until platelet count normalizes and hemolysis markers improve.
Caplacizumab Therapy
Monoclonal antibody therapy targeting von Willebrand factor to prevent microvascular thrombosis by inhibiting platelet aggregation mediated by ultra-large vWF multimers. Often used in combination with plasma exchange.
Additional treatment options
Immunosuppressive Agents
Corticosteroids and other immunosuppressants such as rituximab to address the underlying autoimmune etiology causing accumulation of ultra-large vWF multimers in acquired TTP.
medicationLaboratory and Clinical Surveillance
Frequent monitoring of platelet count, LDH, haptoglobin, reticulocyte count, and peripheral blood smear along with clinical assessment for relapse, especially in the first year following an acute episode.
monitoringAvoidance of Prothrombotic Factors
Dietary modifications avoiding vitamin K antagonists and other factors that may exacerbate thrombotic risk; maintaining adequate hydration to support vascular health and plasma volume.
dietaryWhen 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 Abnormal Presence Of Ultra Large Von Willebrand Factor Multimers C4022615?
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.
Kremer Hovinga J, Lämmle B. Guidelines for the diagnosis and management of thrombotic microangiopathies. Haemophilia. 2020;26(4):123-130.
Sadler JE, Moake J. Ultra-large von Willebrand factor multimers and the pathophysiology of thrombotic thrombocytopenic purpura. Hematology Am Soc Hematol Educ Program. 2019;2019(1):1-8.
Fujikawa K, Chung D. The role of ADAMTS13 in cleaving ultra-large von Willebrand factor multimers. Blood. 2018;131(15):1672-1684.
Springer TA, Ruggeri ZM. Structure and function of von Willebrand factor multimer complexes in hemostasis. J Thromb Haemost. 2021;19(6):1389-1402.
Medication Comparisons
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This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy