A 12-year-old girl was seen in the emergency room, with complaints of heavy bleeding per vaginum, since last 15 days, along with dysmenorrhea. She was referred to us from basic health unit. According to her mother, this was her first menstrual period. She had 7 siblings, 4 sisters, and 3 brothers. Her two elder sisters had normal, regular periods. Her past history was significant for bruises, epistaxis, and bleeding from gums from the age of three years. She was never transfused blood for these complaints, but did receive medical attention for the above complaints of epistaxis and bleeding from gums. One of her cousins had also died at the age of eight years, from uncontrollable hemorrhage from mouth and nose. Her maternal grandfather also died of intracranial hemorrhage, but there was no definitive diagnosis in both of the above deaths.
At the onset of menarche, she suffered from heavy blood loss, with passage of blood clots. The amount of blood loss resulted in transfusion of one unit of blood. She was also kept on injectable tranexemic acid. The bleeding decreased initially for 48 hours, but was later followed by heavy bleeding per vaginum. At the onset of second episode of heavy vaginal bleeding, she was referred to Civil Hospital Karachi, for further management.
General examination revealed extreme pallor, with blood pressure of 90/60
hg. Physical examination was unremarkable. Local examination revealed soiling of perineum, with blood and blood clots. Her hemoglobin concentration was 6
gm/dL, platelets count was 200,000, and prothrombin and partial thromboplastin time was normal. Her pelvic ultrasound was normal, with evidence of hematocolpos. She was started on injectable tranexemic acid 500
mg eight hourly, along with tab norethisterone 5
mg, three times daily. A single injection of depot medroxy progesterone 150
mg was also given. She was transfused four units of packed red blood cells. Her bleeding continued, despite the above measures. Her factor VIII level was assessed to rule out vWD and was found to be 150%. On the seventh day of her admission, her hemoglobin dropped to 3.8
gm/dL, with platelet count of 178,000. At this point we decided to perform platelet aggregation studies. Reports showed the following: ADP 18.1 (76.36–106.84%), collagen 20.4 (59–102%), epinephrine 24.1 (73.7–109.3%), ristocetin 59 (70.4–111.2%), and arachidonic acid 12.9 (55–127%). A diagnosis of Glanzmann's thromboasthenia was made. She was transfused with one mega unit (single-donor apheresis) of platelets then bleeding slowed for 6 hours and started again. She further received 4 more doses of platelets, but there was no improvement in bleeding. At this point, a decision was made for rFVIIa. She was given rFVIIa in a dose of 120μ
g/kg, dose repeated after 4 hours; bleeding slowed down a bit but continued. Subsequent two doses of 270μ
g/kg were given during next 24 hours. Bleeding decreased for 48 hours, but continued, and her hemoglobin again dropped. A decision for uterine tamponade was taken. Examination under anesthesia was done, and two Foleys catheters of size 10
F were passed in the uterine cavity and were inflated with 30
cc of normal saline to act as tamponade. They were left inside for 48 hours; then, bleeding stopped completely. She stayed in hospital for next few days and was discharged on oral contraceptive pills and ferrous sulphate.