| 본문 : An 82-year-old woman with a history of ET, diabetes mellitus and hypertension presented with ocular pain, bleeding and decreased visual acuity of the left eye. She was diagnosed with JAK2 V617F-positive ET 3 years prior, and had been treated with hydroxyurea and anagrelide. She had stopped taking her medication 3 months prior to presentation on her own. She was taking aspirin and cilostazol due to a thromboembolic event in her fingers.
On initial examination, her best corrected visual acuity was 20/100 in the right eye, and light-perception in the left eye. There was bruising around both eyelids and moderate swelling on the upper and lower left eyelids. Her left cornea was not identified because of severe hemorrhagic conjunctival swelling and necrotic conjunctival tissues (Fig. 1A). Hematologic studies revealed a markedly increased platelet count (1,270,000/µL), but other coagulation tests including prothrombin time and activated partial thromboplastin time were normal.
A computed tomography (CT) scan showed a relatively well-defined homogenous mass-like lesion in the left subconjunctival and intraconal space (Fig. 1B). Hemorrhage was suspected, but the conjunctival lesion was necrotic. So, incisional biopsy was performed to rule out any malignancy. Incisional biopsy of the left conjunctival tissue showed acute inflammation with necrosis, and vascular ectasia with thrombosis and hemorrhage (Fig. 1C). After 10 days of treatment with hydroxyurea and anagrelide, her platelet count decreased to a normal level. Periorbital swelling and conjunctival hemorrhage decreased markedly (Fig. 2). Cornea was identified, and appeared slightly edematous. However, anterior chamber was clear. Applanation tonometry was 15 mmHg in the right eye, and 13 mmHg in the left eye. Her best corrected visual acuity improved to 20/400 in the left eye. Hertel measurement results were 10 on the right and 11 on the left with a base of 106. Fundus examination revealed moderate non-proliferative diabetic retinopathy, but there were no optic disc abnormalities or evidence of vascular occlusive disease in either eye. The patient refused any further examination including fluorescein angiography and visual field examination. She was discharged with improved symptoms but was lost to follow-up.
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