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Acquired von Willebrand syndrome
Other Resources UpToDate PubMed

Acquired von Willebrand syndrome

Contributors: Kevin Varghese, Paritosh Prasad MD
Other Resources UpToDate PubMed

Synopsis

Acquired von Willebrand syndrome (AvWS) occurs due to the depletion of circulating von Willebrand factor (vWF). Loss of circulating vWF interferes with primary hemostasis, allowing for mucocutaneous bleeding. The etiology is multivariate and centralized on the plasma clearance of vWF. Mechanisms include underproduction, antibodies against vWF, cell uptake, mechanical degradation, or increased proteolysis. For this reason, AvWS is almost always associated with an underlying disorder. According to the International Society of Thrombosis and Haemostasis (ISTH), the most common underlying cause of AvWS is a lymphoproliferative disorder. Other etiologies include hematologic malignancies, solid organ malignancies, hypothyroidism, cardiac valvular disease (aortic or mitral regurgitation, aortic stenosis, or mechanical valve replacement), and autoimmune disorders, among others.

AvWS is a rare condition. It most commonly presents in an elderly patient with no past or family history of bleeding; this may be partially attributed to elderly patients having higher prevalence of an underlying malignancy. Because this condition is exceedingly rare, there is no specific sex, race / ethnicity, or socioeconomic status noted to be disproportionately affected in the literature.

Typical signs include the sudden onset of mucocutaneous bleeding in someone with no personal or family history. Examples include epistaxis, ecchymosis, menorrhagia, and gastrointestinal tract bleeding, among others. Another possible symptom includes bleeding diathesis, which is seen in elderly patients. The ISTH notes that patients with an underlying lymphoproliferative disorder may be at risk for increased severity of bleeding, but mortality secondary to hemorrhage is rare. Patients may present with a history of a blood dyscrasia such as essential thrombocythemia or polycythemia vera and a propensity toward bleeding. Among these populations, younger age was a predictor for AvWS.

Predisposing medical history includes hypothyroidism, leading to underproduction of vWF. Lymphoproliferative disorders such as monoclonal gammopathy of unknown significance, multiple myeloma, or non-Hodgkin lymphoma produce inhibitory antibodies against vWF. Connective tissue diseases such as systemic lupus erythematosus are also associated with AvWS. Myeloproliferative disorders such as essential thrombocythemia, polycythemia vera, or chronic myelogenous leukemia result in the uptake of vWF into malignant cells. This phenomenon also occurs in other malignancies such as gastric adenocarcinoma or adrenal cell carcinoma. Cardiovascular diseases such as aortic stenosis or other valvular diseases result in the proteolysis of vWF multimers. Patients with cardiac implants, such as left ventricular assist devices, may also share this syndrome. Drugs such as ciprofloxacin, griseofulvin, and valproic acid are implicated in the cell-mediated proteolysis of vWF multimers.

Emergent Care / Stabilization: Emergent care, which may consist of blood transfusions / factor VIII-vWF concentrates, fluids, and pressors as needed for hemodynamic stabilization, is rarely needed for AvWS.

Codes

SNOMEDCT:
234451005 – Acquired von Willebrand's disease
D68.04 – Acquired von Willebrand disease

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Von Willebrand disease – Presents from adolescence in a patient with personal and family history of bleeding.
  • Autoimmune hemolytic anemia (warm, cold) – Coagulation studies of plasma would be normal.
  • Iron deficiency anemia – Measures such as ferritin and iron would be abnormal.
  • Scurvy – Look for perifollicular hemorrhage and dystrophic dentition / gingivae in a patient with poor nutrition.
  • Acquired hemophilia – Use the abnormal prothrombin time (PT) or partial thromboplastin time (PTT) to differentiate.

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:10/04/2022
Last Updated:10/20/2022
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Acquired von Willebrand syndrome
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A medical illustration showing key findings of Acquired von Willebrand syndrome
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