Recurrent hemoperitoneum secondary to haemorrhage from the corpus luteum unmasks factor V deficiency

Abstract

Factor V deficiency is a rare autosomal recessive coagulation disorder. We report a case with inherited factor V deficiency presenting as life-threatening recurrent hemoperitoneum, following bleeding from ruptured corpus haemorrhagicum. Prolonged prothrombin and activated partial thromboplastin times, normal thrombin time and a normal platelet count pointed towards a disorder of coagulation. Mixing studies with factor V deficient plasma and coagulation factor assay revealed markedly reduced plasma factor V clotting activity. The management included blood, plasma and tranexamic acid. Family screening revealed low factor V levels in her parents. Although her brother had significant Factor V deficiency and epistaxis, he did not need hospitalization or replacement, indicating the varied manifestation of this bleeding defect in this family.

Corpus luteum hemorrhage with acquired hemophilia A: a case report and literature review

“…Coagulative disorders, which may also lead to abnormal uterine bleeding (AUB), require differential diagnosis which may cause impaired coagulative status[ 3 ]. Many cases of CL hemorrhage have been reported in patients with von Willebrand disease type 1, 2A, 3 [ 4 – 10 ], afibrinogenemia [ 11 – 14 ], Glanzmann’s thrombasthenia, hemophilia A [ 15 ], hemophilia B, deficiency of factor X, factor V and factor XIII, and in patients receiving anticoagulant therapy for antiphospholipid antibody syndrome (APS) [ 16 – 21 ]. However, we haven’t found any report about CL hemorrhage in patient with AHA (Table 1 ).…”

“…Two episodes of hemoperitoneum from luteal cyst rupture Both episodes removed the ruptured cyst by surgery Khamashta et al [ 18 ] Antiphospholipid antibody syndrome Severe ovarian hemorrhage during warfarin treatment Yamakami et al [ 17 ] Antiphospholipid syndrome Three patients had severe hemorrhagic CL while receiving warfarin treatment All of them required prompt blood transfusion and emergency surgery Castellino et al [ 16 ] Antiphospholipid syndrome Two episodes of ovarian hemorrhage while receiving oral anticoagulation One required oophorectomy Singh et al [ 20 ] Factor XIII deficiency 13 Years old. CL hemorrhage Laparoscopic surgery with blood support Badyal et al [ 21 ] Factor V deficiency 19 Years old. Recurrent CL hemorrhage three episodes Each episode required surgery and blood support Schneider et al [ 14 ] Congenital afibrinogenemia 22 Years old.…”

The predicting factors for indication of surgery in patients with hemoperitoneum caused by corpus luteum cyst rupture

“…Previous reports published the cases of hemoperitoneum caused by CLCR with various coagulopathies. Coagulation disorders included aplastic anemia 11 , 12 , factor V deficiency 13 , factor XIII deficiency 14 , congenital hypofibrinogenemia 15 , and immune thrombocytopenic purpura (ITP) 16 . There was a study in the conservative group using tranexamic acid as well as fluids and blood transfusion 7 .…”