CAJ | 학술논문

目的：对治疗阶段并发大脑后部可逆性脑病综合征（PRES）的血液病患儿的临床资料进行分析，以提高对该病的认识。方法对我院3例血液病患儿治疗过程中并发PRES的发病时间、临床表现、影像特征及治疗转归等进行回顾性分析。结果2例急性淋巴细胞白血病患儿在诱导化疗阶段发生PRES，1例重型β地中海贫血患儿在亲缘性HLA全相合外周血造血干细胞移植术后4个月发生PRES。3例患儿的临床表现均为头痛、视觉障碍、高血压、痫性发作、精神异常及昏迷。3例患儿头颅MRI均显示顶枕叶对称性受累，其中1例患儿的MRI示双侧额颞部亦广泛受累。2例患儿脑病症状均短期内缓解，随访亦无神经系统异常表现；1例经多种抗癫痫药物治疗，随访2年仍有频繁癫痫发作。结论化疗药物和高血压是本组PRES病例发病的重要因素；PRES并不完全可逆，部分可见难治性癫痫等后遗症；及时行头颅MRI检查以早期诊治，也许能够避免永久性脑损伤。
목적：대치료계단병발대뇌후부가역성뇌병종합정（PRES）적혈액병환인적림상자료진행분석，이제고대해병적인식。방법대아원3례혈액병환인치료과정중병발PRES적발병시간、림상표현、영상특정급치료전귀등진행회고성분석。결과2례급성림파세포백혈병환인재유도화료계단발생PRES，1례중형β지중해빈혈환인재친연성HLA전상합외주혈조혈간세포이식술후4개월발생PRES。3례환인적림상표현균위두통、시각장애、고혈압、간성발작、정신이상급혼미。3례환인두로MRI균현시정침협대칭성수루，기중1례환인적MRI시쌍측액섭부역엄범수루。2례환인뇌병증상균단기내완해，수방역무신경계통이상표현；1례경다충항전간약물치료，수방2년잉유빈번전간발작。결론화료약물화고혈압시본조PRES병례발병적중요인소；PRES병불완전가역，부분가견난치성전간등후유증；급시행두로MRI검사이조기진치，야허능구피면영구성뇌손상。
Objective To analyze the clinical data of therapy-related posterior reversible encephalopathy syndrome (PRES) in three children with hematologic disease to understand this disease ulteriorly. Methods Retrospectively analyzed the clinical manifestations,magnetic resonance imaging (MRI) scanning of the brain and neurological outcomes of three cases of PRES at our hospital. Results Two children got PRES during acute lymphoblastic leukemia induction chemotherapy,and one children with majorβ-thalassemia got PRES after hematopoietic stem cell transplantation. All cases had headache,visual distur-bance,hypertension,seizures,mental anomaly and coma.The cranial MRI results showed all three cases were bilateral symmetri-cal in parietal and occipital lobes,and one case with extensive involvement in bilateral fontotemporal lobe in addition. Two cases showed complete recovery after symptomatic treatment during the acute stage. One case was treated by variety of anti-epileptic agents subsequently developed refractory epilepsy after two years. Conclusion Chemotherapeutics and hypertension are im-portant for the development of PRES in these cases. PRES is not fully reversible,status epilepticu during acute stage will cause refractory epilepsy. Early diagnosis by cranial MRI and adequate treatment by withdrawal of potential offending factors are im-portant to prevent permanent neurological deficit.