Frequency and Development of CNS Involvement in Chinese Children With Hemophagocytic Lymphohistiocytosis
Frequency and Development of CNS Involvement in Chinese Children With Hemophagocytic Lymphohistiocytosis
复制标题
DOI：
10.1002/pbc.22239
复制
发表时间：
2010-03-01
影响因子：
3.2
通讯作者：
Shen, Kunling
中科院分区：
文献类型：
--
作者：
Yang, Shuang;Zhang, Li;Shen, Kunling
背景。我们研究了嗜血球性淋巴组织细胞增多症（HLH）患者中枢神经系统（CNS）受损伤的特点、频率和预后。过程。对2004年1月至2008年8月在本中心治疗的92例HLH患儿的神经系统表现进行前瞻性评估；82例（89%）有相关的病毒感染（69例爱泼斯坦-巴尔病毒），1例脓胸，其余9例未发现相关疾病。治疗前，所有患者均接受脑脊液（CSF）评估、脑计算机断层扫描（CT）和/或磁共振成像（MRI）检查。结果。诊断时，43例（47%）患儿有中枢神经受累。12例患者（13%）有神经系统症状，包括癫痫发作、共济失调、昏迷、脑神经麻痹和偏瘫。所有患者在治疗8周后均有所改善，但1例后来出现进行性神经症状，6例因进行性全身症状和/或其他原因停止治疗。15例患者脑脊液异常，治疗6周后全部恢复正常。36例（39%）患者有神经影像学异常；其中仍在治疗5例，失访15例，治疗结束后随访16例。在这16个病人中，12个好转了，3个没有变化，我进步了。在治疗结束后随访的21例中枢神经系统受累患儿中，10例完全恢复，10例改善（3例仍有神经放射学异常），1例临床和神经放射学进展。结论。本文报道的大多数患者患有继发性HLH，由于HLH中常累及中枢神经系统，因此建议所有HLH患者在诊断时进行脑MRI检查。临床和脑脊液异常通常在治疗8周内改善，但CT/MRI异常恢复正常的速度较慢且频率较低。儿科血癌2010；54:408-415。(C) 2009 Wiley-Liss, Inc。
Background. We investigated the characteristics, frequency, and prognosis of central nervous system (CNS) involvement in patients with hemophagocytic lymphohistiocytosis (HLH). Procedure. Neurological manifestations were prospectively assessed in 92 children with HLH treated from January 2004 to August 2008 at Our center; 82 (89%) had associated viral infections (69 Epstein-Barr virus), one empyema, while no associated disease was identified in the remaining nine. Prior to treatment, all underwent cerebrospinal fluid (CSF) evaluation, brain computer tomography (CT) and/or magnetic resonance imaging (MRI). Results. At diagnosis, 43 (47%) children had CNS involvement. Twelve patients (13%) had neurological symptoms, including seizures, ataxia, coma, cranial nerve palsy, and hemiplegia. All patients improved after 8 weeks of therapy, but one later developed progressive neurological symptoms and six discontinued therapy due to progressive systemic symptoms and/or other reasons. Fifteen patients had CSF abnormalities that all normalized completely after 6 weeks of treatment. Thirty-six patients (39%) had neuroradiological abnormalities; with 5 still under treatment, 15 lost to follow-up, and 16 followed after completion of therapy. Of these 16, 12 improved, 3 were unchanged, and I progressed. Among all 21 children with CNS involvement followed after completion of therapy, 10 recovered completely, 10 improved (3 had remaining neuroradiological abnormalities), and I progressed clinically and neuroradiologically. Conclusion. Most patients reported here Suffered from secondary HLH and since CNS involvement is frequent in HLH, brain MRI at diagnosis is recommended in all HLH patients. Clinical and CSF abnormalities often improved within 8 weeks of therapy, but CT/MRI abnormalities normalized more slowly and less frequently. Pediatr Blood Cancer 2010;54:408-415. (C) 2009 Wiley-Liss, Inc.