CAJ | 학술논문

目的：总结成人噬血细胞淋巴组织细胞增生症（HLH）患者中枢神经系统病变的临床表现、实验室检查及头颅影像学特点。方法回顾分析2例出现中枢神经系统症状的成人 HLH 患者的临床表现、脑脊液检查结果、中枢神经系统影像学特点及治疗、转归。结果①无论是原发性还是继发性成人 HLH 患者，均可能出现中枢神经系统受累；②HLH 的神经系统临床表现多样，可以表现为癫痫发作、肢体瘫痪、意识障碍等；③脑脊液检查可见蛋白增高；④头颅影像学可以出现广泛脑白质病变或基底节区异常信号。结论成人 HLH 患者可以出现中枢神经系统受累，其临床表现、脑脊液检查、头颅影像学改变呈现多样性。
목적：총결성인서혈세포림파조직세포증생증（HLH）환자중추신경계통병변적림상표현、실험실검사급두로영상학특점。방법회고분석2례출현중추신경계통증상적성인 HLH 환자적림상표현、뇌척액검사결과、중추신경계통영상학특점급치료、전귀。결과①무론시원발성환시계발성성인 HLH 환자，균가능출현중추신경계통수루；②HLH 적신경계통림상표현다양，가이표현위전간발작、지체탄탄、의식장애등；③뇌척액검사가견단백증고；④두로영상학가이출현엄범뇌백질병변혹기저절구이상신호。결론성인 HLH 환자가이출현중추신경계통수루，기림상표현、뇌척액검사、두로영상학개변정현다양성。
Objective To summarize the characteristics of central nervous system involvement in adult patients with hemophagocytic lym-phohistiocytosis(HLH). Methods Clinical,radiological,and cerebrospinal fluid(CSF)data of two HLH patients with central nervous system involvement were analyzed. Results ①Patients with HLH,primary as well as secondary form,can developed central nervous system symptoms.②Neurological presentations of central nervous system involvement are highly variable,including seizures,paralysis or disturbance of conscious-ness. ③Elevated protein content could be found in CSF analysis. ④Magnetic resonance imaging(MRI)of the brain revealed extensive areas of abnormal signal in white matter and basal ganglia region. Conclusion Central nervous system involvement in adult hemophagocytic lymphohistio-cytosis is common. Vone marrow transplant appears to be the available treatment procedure to reduce the complications.