발작성 야간혈색뇨증 환자의 빈혈과 동반된 유두부종 1예

• 영문명: Papilledema Associated with Anemia in a Patient with Paroxysmal Nocturnal Hemoglobinuria
• 발행기관: 대한안과학회
• 저자명: 우경진 김상국,Kyeong Jin Woo, M.D., Sang Kook Kim, M.D.
• 간행물 정보: 『대한안과학회지』Ophthalmological Society,volume48,number10, 1438~1444쪽, 전체 7쪽
• 주제분류: 의약학 > 기타의약학
• 파일형태: PDF
• 발행일자: 2007.10.31

영문 초록

Purpose: To report a case of bilateral optic disc edema associated with hemolytic anemia and paroxysmal nocturnal hemoglobinuria (PNH). Methods: A 51-year-old woman visited our ophthalmologic clinic complaining of metamorphopsia. Twenty eight years ago, she had been diagnosed with PNH and hemolytic anemia and had received blood transfusion on an irregular basis. The best corrected visual acuity was initially 0.5 in the right eye and 1.0 in the left eye. Light reflex was intact and no afferent pupillary defect was found. Fundus examination revealed severe optic disc swellings with indistinct margins in both eyes. Papillary and peripapillary retinal hemorrhages were also present. Results: A visual field test revealed the enlarged physiologic scotoma in both eyes. Fluorescein angiograms showed hyperfluorescence of the optic disc and blocked fluorescence due to the papillary hemorrhages. Optical coherence tomograms of the optic disc showed the loss of physiologic disc cupping and severe elevation. There was no evidence of an intracranial lesion upon brain magnetic resonance imaging. These findings were compatible with optic disc edema associated with anemia and the management was oriented towards the anemia. At the 2-months follow-up, the best corrected visual acuity of both eyes had improved to 1.0 and optic disc edema markedly decreased. However, the patient`s overall physical condition deteriorated and she expired due to dyspnea and hepatic coma.

키워드

Anemia, Optic disc edema, Paroxysmal nocturnal hemoglobinuria