CASE PRESENTATION:
A 12-year-old female with a known history of GT presented to the
Emergency Department (ED) with a chief complaint of HMB. She was
diagnosed with GT in infancy in setting of extensive bruising, recurrent
mucosal bleeding, and persistent gastrointestinal bleeding. Prior to
menarche, she had recurrent episodes of epistaxis and an episode of deep
tissue hematoma that was managed outpatient with aminocaproic acid. She
had two hospital admissions secondary to gingival and dental bleeding
requiring treatment with intravenous recombinant rFVIIa and a tooth
extraction. She had no need for major surgical procedures to date. She
was followed by Pediatric Hematologist for her condition but had not yet
been evaluated by a Pediatric Gynecologist prior to menarche due to
limited access.
Prior to presentation to the ED, she had experienced menarche followed
by 24 days of HMB refractory to high dose oral contraceptive taper
(Norgestimate-Ethinyl Estradiol 0.25mg-25mcg three times daily for three
days, twice daily for two days, and daily thereafter). As bleeding
continued, she was brought to the ED for further evaluation and admitted
for management of acute blood loss anemia with a team of Pediatric
Hematologists and Gynecologists.
Evaluation included a transabdominal pelvic ultrasound that revealed a
thin endometrial stripe of 5 millimeters. She was started on intravenous
estrogen therapy (intravenous conjugated estrogen 25 mg every four
hours) and transitioned to oral estrogen (conjugated estrogen 2.5mg
every 4 hours) for a total of 48 hours. During this initial phase, the
patient required 5 units of packed red blood cells due to acute blood
loss anemia. Upon improvement of her vaginal bleeding similar to a light
menses, she was transitioned to daily oral progesterone (Norethindrone
Acetate 10mg twice daily). Unfortunately, her HMB soon returned. Due to
persistent bleeding refractory to the aforementioned hormonal therapy,
she received leuprolide acetate (intramuscular 11.25 mg once) to
suppress the HPO axis and induce an hypoestrogenic state. At this time,
she was continued on oral progesterone (Norethindrone acetate 10mg twice
daily) and aminocaproic acid to quell the ongoing bleeding and allow for
leuprolide acetate to take therapeutic effect. Five days after
administration of leuprolide acetate, patient achieved amenorrhea and
was discharged.
Patient had several weeks without vaginal bleeding and a norethindrone
taper was attempted (5mg daily for a week, 2.5mg for a week, then 2.5mg
every other day for a week). However, due to interval return of vaginal
bleeding, a transabdominal pelvic ultrasound was repeated and revealed
an 11mm endometrial stripe. The norethindrone and aminocaproic acid was
resumed. She continued treatment with intramuscular leuprolide acetate
and norethindrone “add-back” therapy for a total of six months with
successful amenorrhea. She was then transitioned to continuous combined
oral contraceptive (Norethindrone-ethinyl estradiol 1.5mg-30mcg once
daily) as the leuprolide acetate treatments were concluded. Multiple
attempts were made to taper the norethindrone “add-back” but bleeding
returned. A final transabdominal pelvic ultrasound revealed a 5 mm
endometrial stripe and she has been maintained with continuous combined
oral contraceptive and additional norethindrone as needed without
recurrence of HMB.