파라단백혈증성각막병증(paraproteinemic keratopathy)의 2예 = Two Cases of Paraproteinemic Keratopathy

다국어 초록 (Multilingual Abstract)

Purpose: To report two cases of paraproteinemic keratopathy (PK).
Case summary: A 57-year-old man visited the ophthalmology clinic complaining of decreased visual acuity since 1 year earlier.
White crystalline deposits were observed on both corneas and the best corrected visual acuity (BCVA) of both eyes was 1.0.
After 3 months, the BCVA decreased to 0.8 in the right and 0.6 in the left. Increased corneal deposits were observed. To rule out PK, his medical records were checked. The patient had been diagnosed with lymphoplasmacytic lymphoma 3 years earlier.
Diagnosing PK, the patient was referred back to the Hemato-Oncology department. After 3 months of chemotherapy, the corneal deposits decreased and BCVA returned to 1.0. A 69-year-old woman presented complaining of decreased visual acuity for 2 years. The uncorrected visual acuity (UCVA) was 0.4 in the right and finger count at 30 cm in the left. Brown deposits were observed on both corneas and lenses. The serum copper level was elevated. She was referred to the internal medicine department and diagnosed with multiple myeloma. After treatment, the laboratory results returned to normal, but the UCVA decreased to hand motion in the left. A left white cataract was noted and cataract surgery was performed. 1 month postoperatively, the BCVA was 0.9, with no change in the corneal deposits.
Conclusions: When there are corneal deposits, we should consider PK in the differential diagnosis. In such cases, systemic treatment is the first line. Therefore, diagnosing and referring PK patients to the internal medicine department is crucial.

국문 초록 (Abstract)

목적: 파라단백혈증성각막병증의 2예를 보고하고자 한다.
증례요약: 57세 남자가 1년 전 시작된 양안 시력저하로 안과에 내원하였다. 각막의 흰색 결정성 침착물이 관찰되었으며, 최대교정시력양안 1.0이었다. 3개월 후 시력은 우안 0.8, 좌안 0.6으로 감소하였으며, 침착물도 증가하였다. 림프형질세포성림프종 진단력을 고려하여 파라단백혈증성각막병증 진단하에 혈액종양내과에 의뢰하였고, 치료를 시작하였다. 3개월 후 양안 침착물이 감소하였으며, 양안시력은 1.0으로 호전이 있었다. 69세 여자가 2년 전 시작된 양안 시력저하로 안과에 내원하였다. 각막 및 수정체 전낭의 갈색 침착물이 관찰되었으며, 나안시력은 우안 0.4, 좌안 안전수지였다. 혈청 구리농도의 상승이 확인되어 내과에 의뢰하였고, 다발성골수종이진단되었다. 치료 시작 후 혈액 수치는 정상 범위로 회복되었으나, 좌안 시력은 안전수동으로 감소되었고 백색백내장 진단하에 백내장수술을 시행하였다. 술 후 각막침착은 비슷하나 시력은 0.9로 상승하였다.
결론: 각막의 침착물 발견 시 전신 질환에 의한 각막병증을 고려해야 하며, 파라단백혈증성각막병증은 내과 치료가 안과 치료에 선행되어야 한다. 파라단백혈증에서 각막병증 발병 시 내과 의뢰를 적극적으로 고려해야 한다.

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