噬血细胞性淋巴组织细胞增生症的中枢神经系统受累：单中心经验。
Central nervous system involvement in hemophagocytic lymphohistiocytosis: a single-center experience.
机构信息
Department of Pediatric Hematology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Neurology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Radiology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Immunology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia.
Department of Pediatric Hematology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Radiology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia; Department of Immunology, Mother and Child Health Institute, Medical Faculty, University of Belgrade, Serbia.
出版信息
Pediatr Neurol. 2014 Mar;50(3):233-7. doi: 10.1016/j.pediatrneurol.2013.10.014. Epub 2013 Nov 1.
BACKGROUND
Hemophagocytic lymphohistiocytosis is a rare multisystem disorder characterized by proliferation and diffuse infiltration multiple organs with histiocytes, including the central nervous system.
PATIENTS AND METHODS
Thirty children diagnosed with hemophagocytic lymphohistiocytosis between 1997 and 2010 were reviewed and analyzed. Central nervous system disease involvement was defined as the presence of neurological symptoms and signs or elevated values of cerebrospinal fluid cells and/or proteins.
RESULTS
Among the 30 patients, 17 (56%) had central nervous system involvement. Fourteen patients (46%) presented with neurological symptoms including seizures, irritability, bulging fontanelle, cranial nerve palsy, or disturbance of consciousness, whereas the remaining three patients developed central nervous system symptoms during the course of the disease. Seventeen patients (56%) had cerebrospinal fluid abnormalities. Neuroradiological studies were performed in nine patients. The most common findings were edema, atrophy, subcortical necrosis, and high signal intensity on T2-weighted magnetic resonance imaging. All patients were treated according to the Hemophagocytic Lymphohistiocytosis-94 and Hemophagocytic Lymphohistiocytosis-2004 protocols. Patients with central nervous system involvement had greater mortality. In prediction of the outcome, the cutoff value for cerebrospinal fluid protein was 470 mg/L. The most common neurological sequela was psychomotor delay.
CONCLUSION
Central nervous system involvement in hemophagocytic lymphohistiocytosis is common and is associated with poor outcome.
背景
噬血细胞性淋巴组织细胞增生症是一种罕见的多系统疾病，其特征为组织细胞增生并弥漫浸润多个器官，包括中枢神经系统。
患者与方法
回顾性分析了 1997 年至 2010 年间确诊的 30 例噬血细胞性淋巴组织细胞增生症患儿。中枢神经系统受累定义为存在神经系统症状和体征或脑脊液细胞和/或蛋白升高。
结果
30 例患儿中 17 例（56%）存在中枢神经系统受累。14 例（46%）患儿出现神经系统症状，包括癫痫发作、易激惹、膨隆的囟门、颅神经麻痹或意识障碍，而其余 3 例患儿在病程中出现中枢神经系统症状。17 例（56%）患儿存在脑脊液异常。9 例患儿进行了神经影像学检查。最常见的发现是水肿、萎缩、皮质下坏死和 T2 加权磁共振成像上的高信号强度。所有患儿均根据噬血细胞性淋巴组织细胞增生症-94 方案和噬血细胞性淋巴组织细胞增生症-2004 方案进行治疗。中枢神经系统受累患儿死亡率更高。预测预后时，脑脊液蛋白的截断值为 470mg/L。最常见的神经后遗症是精神运动发育迟缓。
结论
噬血细胞性淋巴组织细胞增生症中中枢神经系统受累常见，且与不良预后相关。
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