A Rare Case of Inherited Factor-II Deficiency Causing Life-Threatening Menorrhagia
A 22-year-old woman presented to our emergency department with severe menstrual bleeding since 12 days, with signs of congestive cardiac failure. Her history revealed that she had menorrhagia since menarche and had severe postpartum hemorrhage (PPH) in both her pregnancies requiring blood transfusions. In the last pregnancy, she required an emergency subtotal hysterectomy. The patient continued to have regular menstrual cycles with heavy bleeding even after subtotal hysterectomy. She would bleed for 6 to 7 days every 28-30 days. However, she did not have abnormal bleeding manifestations from any other sites in the body or any family history of bleeding disorder.\nOn examination, she was conscious, oriented, and very pale. On per speculum examination cervix appeared normal. Uterus was not felt on bimanual examination. Laboratory investigations revealed: Hemoglobin = 3 g/dl%, normal bleeding time, prolonged prothrombin time: test-44.5 s, control-17.2 s, prolonged activated Partial Thromboplastin Time: test-41.4 s, control-28.4 s and normal thyroid function tests and liver function tests. Ultrasound examination revealed the presence of bilateral normal ovaries and a small uterine stump measuring 3 × 3 cm. Other organs were normal. As patient had signs of congestive cardiac failure due to severe anemia, three units of packed cell volume under the cover of diuretics was given and the patient had signs of improvement.\nHer history of menorrhagia since menarche and PPH in both her pregnancies and prolonged prothrombin time and activated Partial Thromboplastin Time was suggestive of an underlying bleeding disorder as the cause of menorrhagia.\nA complete coagulation workup identified her to have deficient prothombin activity of 26%, with all other coagulation factors and platelet functions to be normal. Coagulation workup was as follows: Platelets: 5,99,000/mm3, BT: 3 min, platelet aggregometry: normal response to Ristocetin, ADP, epinephrine, arachidonic acid and collagen. PT: Pt: 18.5 s, normal range: 9.7-13.3 s, APTT: pt: 44.5 s, normal range: 23.8-37.4 s, F II: 26%, F V: 153.6%, F IX: 187%, FX: 73.2%, F VIII: C: 171.6%, Ricof: 122%, Fibrinogen: 364.6 mg%, Lupus Anticoagulant: negative, Clot retraction: Good, Factor XIII activity: Normal.\nHence, bleeding disorder was the cause of life-threatening menorrhagia.\nAfter the diagnosis of factor II deficiency, the patient was put on continuous low dose oral contraceptive pill containing levonorgestrol 100 ug and ethinyloestradiol 20 ug to suspend her menstrual cycle. The patient has been coming for regular follow-up and had no further episodes of bleeding per vagina or from any other sites in the body.