Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis.
Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis.
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DOI：
10.5045/kjh.2012.47.4.273
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发表时间：
2012-12
期刊：
影响因子：
--
通讯作者：
Koh KN
中科院分区：
文献类型：
--
作者：
Kim MM;Yum MS;Choi HW;Ko TS;Im HJ;Seo JJ;Koh KN
噬血细胞性淋巴组织细胞增多症是一种罕见的多系统疾病，常累及中枢神经系统。我们根据中枢神经系统受累的程度比较了HLH患者的临床特点、治疗和预后。对1995年1月至2011年8月在峨山医学中心就诊的50例HLH患者的临床表现、实验室检查资料、治疗方法及转归进行了回顾分析。中枢神经系统受累定义为出现神经症状或脑脊液中白细胞(WBC)计数升高。在这50例患者中，23例(46%)发展为中枢神经系统疾病。在中枢神经系统疾病患者中，19例有神经系统症状，包括癫痫发作、意识改变、面瘫、构音障碍和吞咽困难。4名患者的脑脊液白细胞计数升高，没有神经系统症状。12例患者有异常的脑成像结果，包括T2加权磁共振成像(MRI)上的高信号病变，脑室增大，出血，萎缩和软脑膜强化。与无中枢神经系统疾病的患者相比，中枢神经系统疾病患者的铁蛋白、天冬氨酸转氨酶(AST)和丙氨酸氨基转移酶(ALT)水平降低，5年生存率降低。中枢神经系统受累在HLH患者中很常见。总体而言，有中枢神经系统疾病的患者比没有中枢神经系统受累的患者预后更差。为了改善预后，医生必须仔细监测HLH患者的神经表现，并对CNS疾病患者进行适当的强化化疗。
Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem disorder that frequently involves the central nervous system (CNS). We compared the clinical characteristics, treatment, and prognosis of patients with HLH according to the degree of CNS involvement. The clinical manifestations, initial laboratory data, treatment, and outcomes for 50 patients diagnosed with HLH and treated at Asan Medical Center between January 1995 and August 2011 were retrospectively reviewed and analyzed. CNS involvement was defined as the presence of neurological symptoms or an elevated white blood cell (WBC) count in the cerebrospinal fluid (CSF). Among these 50 patients, 23 (46%) developed CNS disease. Among patients with CNS disease, 19 had neurological symptoms, including seizures, altered consciousness, facial palsy, dysarthria, and dysphagia. Four patients had elevated CSF WBC counts without neurological symptoms. Twelve patients had abnormal brain imaging results, including high signal intensity lesions on T2-weighted magnetic resonance imaging (MRI) findings, ventriculomegaly, hemorrhage, atrophy, and leptomeningeal enhancement. Patients with CNS disease had lower ferritin, aspartate aminotransferase (AST), and alanine aminotransferase (ALT) levels as well as reduced 5-year survival as compared to those without CNS disease. CNS involvement is common among patients with HLH. Overall, patients with CNS disease achieve poorer outcomes than patients without CNS involvement. To improve outcomes, physicians must carefully monitor the neurological manifestations in patients with HLH and administer the appropriate course of intensified chemotherapy to patients with CNS disease.