발작성야간혈색뇨증환자의빈혈과동반된유두부종 1 예 1438
Figure 1. Photograph of peripheral blood smear. The erythrocytes are relatively normal in size and in hemoglobin content, but they are insufficient in number. These findings correspond to normocytic normochromic features of hemolytic anemia (Wright-Giemsa stain, 400). Figure 2. Fundus photographs show bilateral disc edema at the first visit. The disc margin is indistinct and papillary and peripapillary retinal hemorrhages are accompanied. 1439
우경진 외 : 빈혈과 동반된 유두부종 Figure 3. Automated Humphrey visual field test demonstrates enlarged physiologic scotoma and irregular peripheral visual field defects. Figure 4. Early (A, B) and late (C, D) phase fluorescein angiograms show continued hyperfluorescence of the disc accompanied blocked fluorescence by papillary and peripapillary hemorrhages. 1440
대 한 안 과 학 회 지 제 48 권 제 10 호 2007년 Figure 5. Optical coherence tomographs of the right (A) and left (B) optic disc show the loss of physiologic disc cupping and elevated optic disc. Figure 6. Orbit (A) and brain (B) MRIs of the patient reveal no evidence of retrobulbar lesion and brain metastasis or hydrocephalus. The superior ophthalmic vein (arrow) is not dilated. 그러나 환자의 전신상태는 더욱 악화되어 호흡곤란 과 간성혼수로 사망하였다. 고 Figure 7. Abdomen CT shows diffuse swelling of the liver (*) and spleen (**) with left portal vein thrombosis (arrow). 찰 두개강내압의 상승과 이로 인한 유두부종은 철결핍 성 빈혈과 동반되어 가장 흔하게 발생하지만, 다른 여 러가지 형태의 빈혈에서도 발생된 예가 보고되고 있 다.4-16 이 증례들 중 대부분은 수혈을 통해 유두부종이 호전된 것으로 보아, 빈혈과 유두부종은 직접적인 연관 이 있음을 짐작할 수 있지만 빈혈과 동반되어 시신경유 두부종이 발생하는 기전에 대해서는 명확히 밝혀진 바 가 없다. 17 Capriles 는 철결핍에 의한 시토크롬계통(cyto 1441
Figure 8. Fundus photographs after 2 months show improved papilledema. 1442
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Papilledema Associated with Anemia in a Patient with Paroxysmal Nocturnal Hemoglobinuria Kyeong Jin Woo, M.D., Sang Kook Kim, M.D. Department of Ophthalmology, Chungbuk National University College of Medicine, Cheongju, Korea 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. J Korean Ophthalmol Soc 48(10):1438-1444, 2007 Key Words: Anemia, Optic disc edema, Paroxysmal nocturnal hemoglobinuria 1444