Recurrent haemoperitoneum in a mild von Willebrand's disease combined with a storage pool deficit

Abstract

Haemoperitoneum secondary to haemorrhagic corpus luteum has been described in severe bleeding disorders such as afibrinogenaemia, type 3 von Willebrand's disease and patients under oral anticoagulation. We have studied one patient who presented three episodes of severe bleeding at ovulation, requiring surgery twice, with the diagnosis of mild von Willebrand's disease and mild storage pool deficiency. Mild von Willebrand's disease (associated with other thrombopathies or coagulopathies) should be considered in this pathology, although physicians would prefer to find a severe haemorrhagic disorder as the underlying condition in these cases.

Von Willebrand Disease

“…There are multiple case reports of women with VWD experiencing hemorrhagic ovarian cysts (11,10,(69)(70)(71)(72)(73). Silwer reported that 9 of 136 women with VWD (6.8%) experienced this problem (19).…”

von Willebrand's disease and menorrhagia: Prevalence, diagnosis, and management

“…Bleeding often recurred and required transfusion in the majority of cases [4]. Massive hemoperitoneum due to rupture of a hemorrhagic corpus luteum is an uncommon but serious complication of vWD [40][41][42][43][44][45]. Patients present with severe midcycle abdominal pain, hypotension, and profound anemia, often requiring surgical exploration and aggressive bloodproduct replacement.…”

Von Willebrand disease and recurrent hematoperitoneum due to the rupture of haemorrhagic ovarian cysts

“…The female patients in reproductive age with von WillebrandÕs disease (VWD) have twice higher incidence of haemorrhagic ovarian cysts in comparison to the general population [1]. The cysts are as a result of excessive bleeding into the corpus luteum at the time of ovulation, and rupture of these cysts may result in hematoperitoneum [1][2][3][4][5][6][7]. Recurrent and excessive hematoperitoneum and secondary increased production of fibrin in the peritoneal cavity can be a major cause of increased formation of pelvic adhesions, external occlusions of the fallopian tubes, and destruction of the ovarian tissue, and could lead to reduced fertility in these patients [1][2][3][4][5][6][7].…”

“…The incidence of VWD in general population is around 1% and the number of cases of hematoperitoneum in female patient with VWD, described in the literature does not reveal their real incidence because majority of the cases remains unrecognized. In five out of eight cases (including our case), the hematoperitoneum occurs in the mild and moderate form of VWD, and in five out of eight cases, the OHCP was used in further management of haemorrhagic cysts [1][2][3][4][5][6][7].…”

“…Furthermore, excessive and recurrent formation of the haemorrhagic ovarian cysts and surgical intervention lead to the destruction of the remaining healthy ovarian tissue. In those patients, the prevention of recurrence of hematoperitoneum is essential to avoid massive and life threatening bleeding and to preserve fertility [1][2][3][4][5][6][7].…”