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再生障碍性贫血合并自身免疫性溶血患者一例,长期口服环孢素,因“确诊再生障碍性贫血9+年,发热3天”入住血液科,予环磷酰胺静脉治疗9天后,突发意识障碍及四肢抽搐,转入神经科ICU治疗,头颅CT:双侧顶叶、枕叶多发斑片状低密度影。头颅MRI:左侧颞叶、双侧额叶、顶叶、枕叶、胼胝氏体、双侧小脑半球及小脑蚓部多发斑片状长T1、长T2信号,边界不清,FLAIR像呈高信号。DWI像:双侧枕叶、右侧小脑半球间散在高信号,考虑诊断为:(1)可逆性后部脑病综合征;(2)再生障碍性贫血;(3)自身免疫性溶血。予停用环孢素及环磷酰胺、脱水降颅压、丙戊酸钠抗癫痫、对症、支持治疗,9天后,症状消失,转入普通病房。复习此病例以学习可逆性脑后部综合征,以免临床上漏诊及误诊。
A case of aplastic anemia combined with autoimmune hemolytic patients, long-term oral cyclosporine, due to “diagnosed aplastic anemia 9+ years, fever 3 days ” admitted to the Department of Hematology, given cyclophosphamide intravenously 9 days after the sudden Unconsciousness and convulsions in the extremities, transferred to Neurology ICU treatment, head CT: bilateral parietal lobe, occipital lobe multiple patchy low density shadow. Head MRI: the long temporal T1 and long T2 signals of left temporal lobe, bilateral frontal lobe, parietal lobe, occipital lobe, corpus callosum, bilateral cerebellar hemispheres, and cerebellar vermis showed unclear border and FLAIR signal. DWI like: bilateral occipital lobe, scattered between the right cerebellar hemispheres high signal, consider the diagnosis: (1) reversible posterior encephalopathy syndrome; (2) aplastic anemia; (3) autoimmune hemolysis. To stop using cyclosporine and cyclophosphamide, dehydration reduced intracranial pressure, sodium valproate antiepileptic, symptomatic, supportive treatment, 9 days later, the symptoms disappeared, transferred to the general ward. Review of this case to learn about reversible posterior cerebral syndrome, so as to avoid clinical misdiagnosis and misdiagnosis.