CAJ | 학술논문

目的探讨白血病患儿造血干细胞移植后眼卡波西肉瘤(Kaposi's sarcoma,KS)的临床特征.方法回顾性分析1例HIV阴性急性淋巴细胞白血病(ALL)患儿非血缘相关外周血干细胞移植(PBSCT)后发生单眼角膜、结膜及巩膜KS的临床资料.结果患儿为高危T细胞型ALL,接受PBSCT后7个月出现右眼视物模糊,缓慢进展为角结膜新生物.病理表现为毛细血管瘤样改变及异型性梭形细胞,结合典型免疫组化及原位杂交人类疱疹病毒8型(HHV8)阳性,诊断为右眼KS.行角结膜巩膜肿物切除及角膜巩膜移植术,予抗病毒、抗感染、促修复、增强免疫力、预防角膜排斥等治疗,右眼保留且有手动视力,原发病未复发,未发生移植物抗宿主病.结论首次报告国内ALL患儿造血干细胞移植后发生与HIV感染无关眼KS.手术及生物治疗对眼KS是一种安全有效的方法.
목적 탐토백혈병환인조혈간세포이식후안잡파서육류(Kaposi's sarcoma,KS)적림상특정.방법 회고성분석1례HIV음성급성림파세포백혈병(ALL)환인비혈연상관외주혈간세포이식(PBSCT)후발생단안각막、결막급공막KS적림상자료.결과 환인위고위T세포형ALL,접수PBSCT후7개월출현우안시물모호,완만진전위각결막신생물.병리표현위모세혈관류양개변급이형성사형세포,결합전형면역조화급원위잡교인류포진병독8형(HHV8)양성,진단위우안KS.행각결막공막종물절제급각막공막이식술,여항병독、항감염、촉수복、증강면역력、예방각막배척등치료,우안보류차유수동시력,원발병미복발,미발생이식물항숙주병.결론 수차보고국내ALL환인조혈간세포이식후발생여HIV감염무관안KS.수술급생물치료대안KS시일충안전유효적방법.
Objective To summarize clinical features of eye Kaposis' sarcoma (KS) in leukemia child after peripheral blood stem cell transplantation (PBSCT).Methods One 13 years-old child with acute lymphoblastic leukemia (ALL) and negative HIV test who developed KS restricted in right conjunctiva,cornea and sclera after successful atlogeneic PBSCT was reviewed retrospectively.Results The child suffered from T cell type ALL.He received immunosuppressive treatment after PBSCT,and had once extensive herpes zoster restricted in skin.Seven months after PBSCT,he had blurred vision with right eye and slowly neoplasm formed in cornea and conjunctiva.Pathological examination confirmed KS with changes like capillary hemangioma,atypical fusiform cell,typical immunochemistry and positive immunofluorescent result of HHV8.He received excision of lump of cornea,conjunctiva,sclera and transplantation of cornea and sclera.Antiviral therapy was given together with anti-infection,prevention of cornea rejection and biotherapy.He kept right eye and hand-move eyesight,survived without GVHD or recurrence of ALL and KS.Conclusion This was the first ocular KS case in ALL child after PBSCT,without correlation with HIV infection.Complete excision combined with biotherapy was safe and effective for single ocular lesions.