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Recurrent hemoperitoneum secondary to haemorrhage from the corpus luteum unmasks factor V deficiency
Rama K Badyal1, Kanupriya Jain, Kavita Mandrelle
1aDepartment of Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh bDepartment of Obstetrics and Gynecology cDepartment of Clinical Hematology, Hemato Oncology and Bone Marrow Transplant Unit dDepartment of Pathology, Christian Medical College & Hospital, Ludhiana, Punjab eDepartment of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
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.
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.
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