Rare von Willebrand case resolves after rectal cancer treatment: Report
Acquired VWS linked to gastrointestinal adenocarcinoma for first time
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The case of a woman whose von Willebrand syndrome (VWS) resolved spontaneously after being treated for rectal cancer, was reported in a recent study.
VWS was detected just before surgery to treat the cancer that starts in the rectum, after blood testing showed an unexplained abnormal clotting result. After preventive treatment to raise the levels of von Willebrand factor, the protein needed in blood clotting that is lacking in von Willebrand disease, she underwent surgery without relevant bleeding.
“To our knowledge, this is the first reported case of [acquired von Willebrand syndrome] secondary to gastrointestinal adenocarcinoma,” researchers wrote. Adenocarcinoma is a type of cancer that starts in gland cells.
The report, “Acquired von Willebrand syndrome in a patient with rectal adenocarcinoma: a case report and review of the literature,” was published in the Journal of Medical Case Reports.
Acquired VWS develops later in life due to another condition
Unlike von Willebrand disease, which is inherited, acquired VWS develops later in life due to another condition or medications that lead to low von Willebrand factor activity levels.
Among the conditions that have been linked to acquired VWS are heart disorders, blood cancers, autoimmune diseases, hypothyroidism (underactive thyroid), and, in rare cases, certain solid tumors.
A team in Switzerland described the case of a woman with newly diagnosed rectal adenocarcinoma who developed acquired VWS.
The woman, age 62, had no family history of abnormal bleeding. Her medical history included hypertension (high blood pressure), high cholesterol, and hypothyroidism. She was scheduled for surgery to remove her tumor, but first underwent neoadjuvant treatment, which included chemotherapy and radiation. Neoadjuvant treatment is given before surgery with the goal of shrinking tumors and helping to improve surgical outcomes.
MRI scans after the treatment showed a slight reduction in tumor volume and reduced size of most nearby lymph nodes.
Just before surgery, blood tests showed that the woman’s blood took longer than normal to clot. Further tests found low levels and reduced activity of von Willebrand factor. Levels of factor VIII, which is stabilized and transported by von Willebrand factor to the site of injury to promote blood clotting, also were low. Taken together, these findings raised the suspicion of acquired VWS.
[Acquired VWS] should be considered in patients with solid tumours who present with unexplained, clinically relevant bleeding or abnormal global coagulation assays.
She received treatment with Wilate, a replacement therapy that provides VWF and FVIII, together with tranexamic acid, a medication used to help prevent bleeding.
The treatment led to a rapid increase in the levels of both clotting factors. The woman then underwent robot-assisted surgery to remove the rectal tumor and create a colostomy, or an opening in the belly where part of the colon is brought through and attached to a pouch outside the body. No major or abnormal bleeding occurred during or after surgery.
Following surgery, she received additional chemotherapy and achieved complete remission of the tumor. One month after completing chemotherapy, testing showed normal von Willebrand factor activity, which was sustained at five months. Overall, no evidence of acquired VWS was found.
According to the researchers, the recovery of von Willebrand factor levels after cancer treatment supported the diagnosis of AVWS secondary to rectal adenocarcinoma.
Overall, this case report highlights how acquired VWS “should be considered in patients with solid tumours who present with unexplained, clinically relevant bleeding or abnormal global coagulation assays,” the authors wrote. “Further studies are needed to elucidate the mechanisms underlying the development of [acquired VWS] and to guide treatment in acute and follow-up settings.”
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