Angiodysplasia as a Serious Problem in Patients with von Willebrand Disease − Case Study

Case Description – Introduction

A 59-year-old man with a known diagnosis of type 2B vWD presents with a short history of melena. Upon examination, his basic vital functions are stable, but laboratory tests showed anemia with a hemoglobin level of 85 g/l (two months ago, during a routine check-up, it was 140 g/l). The man is therefore admitted to the hospital and treated with vWF/FVIII concentrate at a dose of 80 IU/kg at 8-hour intervals. Endoscopic examination performed the next day did not reveal the source of the bleeding. Capsule endoscopy of the GIT revealed bleeding angiodysplasia lesions in the ileum. These lesions were then treated by specialists using argon plasma coagulation. Ten days after admission, the man can be discharged with stable hemoglobin levels and no further clinical signs of ongoing bleeding. After discharge, he continues with daily prophylactic treatment with vWF/FVIII concentrate.

Angiodysplasia and GI Bleeding

GI bleeding is one of the most common reasons for hospitalization in people with vWD related to bleeding. Vascular malformations, especially in the GI tract, are associated with a defect in vWF. They have been described in both congenital and acquired vWD. Some studies suggested that angiodysplasia is associated with the absence of high-molecular-weight multimers (HMWM) of vWF. However, it was later proven that angiodysplasia occurs in various subtypes of vWD, most frequently in type 2A. Angiodysplasia is not only localized in the GI tract, but GI localization poses a significant problem in terms of bleeding.

Regarding the mechanism of angiodysplasia development in vWD, studies have shown that vWF plays a regulatory role in angiogenesis in terms of extracellular and intracellular pathways influencing VEGF signaling.

In a survey conducted in the USA regarding GI bleeding, it was found that angiodysplasia was the cause of 36.5% of bleeding in people with a diagnosis of vWD (compared to the general population, where it was described in only 9.5% of bleeding individuals). It is believed that the true incidence of angiodysplasia in individuals with vWD is underestimated and underdiagnosed, mainly due to the difficulty in proving its existence.

Management of GI Bleeding

In the treatment of GI bleeding in people with vWD, in addition to attempting to locally treat the bleeding lesion, it is important to ensure treatment with desmopressin, vWF/FVIII concentrate, and antifibrinolytics. It is often necessary to administer up to twice the dose of vWF concentrate to achieve effective hemostasis. In complicated situations, intestinal resection is necessary. However, it should not be forgotten that similar lesions may appear in the future, so resection should be kept as minimal as possible.

In preventing recurrence of GI bleeding, the most effective method is regular administration of vWF/FVIII concentrates. Prophylaxis is also recommended for patients with a history of severe bleeding, as per the latest guidelines. Additional substances targeting angiogenesis to influence the development of angiodysplasia have also been tested, with atorvastatin being the most commonly administered.

Case Description – Conclusion

In the following two months, despite prophylaxis, the patient required transfusions of red cell mass and was rehospitalized three times for GI bleeding. As a result, additional treatment with atorvastatin at a dose of 80 mg daily was initiated to influence angiodysplasia. In the three months following the initiation of this treatment, the need for transfusions disappeared, and the frequency of prophylaxis administration decreased from daily to less frequent intervals. A year after starting atorvastatin treatment, the patient is in good condition and has not required hospitalization.

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Source: Chornenki N. L. J., Ocran E., James P. D. Special consideration in GI bleeding in vWD patients. Hematology Am Soc Hematol Educ Program 2022 Dec 9; 1: 624−630, doi: 10.1182/hematology.2022000390.