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Scholars Journal of Medical Case Reports | Volume-14 | Issue-02
Digestive Angiodysplasia Revealing Acquired Type 3 Von Willebrand Syndrome Associated with MGUS: A Case Report
Oumaima Skalante, M. Ababou, S. Oualaalou, M Hachimi Idrissi, S Elbou, A. Ouahid, I Orchi, Z Jeroundi, S Kahouli, K Essahli, H Zahid
Published: Feb. 7, 2026 |
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Pages: 225-228
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Abstract
While congenital von Willebrand disease is common and usually diagnosed early, acquired von Willebrand syndrome (AVWS) remains a rare and often underestimated cause of bleeding disorders in elderly patients. It typically occurs in the absence of personal or family history of bleeding and is most often associated with an underlying condition. The clinical presentation of AVWS is polymorphic, predominantly involving mucocutaneous or gastrointestinal manifestations. Gastrointestinal bleeding, frequently related to angiodysplasias, may be chronic or recurrent and represents a major cause of iron-deficiency anemia and prolonged diagnostic delay. This clinical variability explains the frequent delay in disease identification. Diagnosis relies on a combination of clinical and biological findings demonstrating a deficiency of von Willebrand factor, impaired functional activity, and reduced factor VIII levels. Etiological investigation is essential for appropriate therapeutic management. Severe forms, related to type 3 deficiency, expose patients to potentially life-threatening spontaneous bleeding. Management primarily relies on treatment of the underlying causal condition, which is the only approach capable of achieving sustained improvement in hemorrhagic manifestations. Early recognition of AVWS, particularly in the presence of unexplained angiodysplasias, significantly improves patient prognosis.


