급성 전골수구성백혈병 환자에서 발생한 ATRA 분화증후군 관련 안침범 1예
Differentiation Syndrome with Ocular Manifestations in Acute Promyelocytic Leukemia Patients Treated with All-trans Retinoic Acid
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목적: 급성 전골수구성백혈병(acute promyelocytic leukemia) 환자에서 all-trans retinoic acid (ATRA) 치료 중 발생한 분화증후군관련 안침범 사례를 경험하였기에 이를 보고하고자 한다. 증례요약: 27세 여자 환자는 급성 전골수구성백혈병을 진단받고 ATRA 복용을 하던 중 시야가 노랗게 보이는 증상과 함께 시력저하를 호소하였다. 안저검사상 양안에 미만성으로 퍼져 있는 다수의 드루젠 유사 병변과 빛간섭단층촬영상 다수의 색소망막상피층의 박리가 보였다. 형광안저혈관조영술상 연한 노란색 병변과 대응되는 다수의 점상 과형광과 후기 누출이 확인되었고, 인도사이아닌그린혈관조영술상 다수의 저형광 반점이 보였다. 본 환자는 ATRA를 중단하고 고농도 덱사메타손(Dexamethasone)을 투약 받은 이후 양안 시력이 회복되었고 맥락망막병변 또한 소실되었다. 결론: ATRA 치료를 받는 급성 전골수구성백혈병 환자에서 분화증후군과 연관된 맥락망막병증이 발생할 가능성이 있다. 이는 분화증후군의 전신적 악화를 시사할 수 있으므로 적극적인 진료와 빠른 치료가 필요하다.
Abstract
Purpose: To report a rare case of differentiation syndrome with ocular manifestations in a patient with acute promyelocytic leukemiatreated with all-trans retinoic acid (ATRA). Case summary: A 27-year-old female complained of yellowing of vision and decreased visual acuity during an ATRA medicationcourse for acute promyelocytic leukemia. Bilateral diffuse drusen-like lesions were found at the posterior pole along with multiplepigment epithelial detachment on optical coherence tomography (OCT) scans. Fluorescein angiography showed multiple hyperfluorescentlesions with leakage at the late phase corresponding to drusen-like lesions in fundus photography. Indocyaninegreen angiography revealed multiple hypocyanescent lesions. ATRA treatment was discontinued and replaced with high-dosedexamethasone. Accordingly, the patient experienced a rapid improvement in visual symptoms and the chorioretinal lesions onOCT scans showed marked resolution. Conclusions: Differentiation syndrome-associated chorioretinopathy may occur in patients with acute promyelocytic leukemiatreated with ATRA. Because the occurrence of chorioretinopathy may be associated with systemic aggravation of the ATRA syndrome,preemptive treatment with early detection is required.
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