Hemopathy Center, Beijing Children's Hospital, Capital Medical University, Beijing, China.
Pediatr Blood Cancer. 2010 Mar;54(3):408-15. doi: 10.1002/pbc.22239.
We investigated the characteristics, frequency, and prognosis of central nervous system (CNS) involvement in patients with hemophagocytic lymphohistiocytosis (HLH).
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).
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 1 progressed. Among all 21 children with CNS involvement followed after completion of therapy, 10 recovered completely, 10 improved (3 had remaining neuroradiological abnormalities), and 1 progressed clinically and neuroradiologically.
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.
我们研究了噬血细胞性淋巴组织细胞增多症（HLH）患者中枢神经系统（CNS）受累的特征、频率和预后。
2004 年 1 月至 2008 年 8 月，我们中心对 92 例 HLH 患儿进行了前瞻性评估，这些患儿均有神经系统表现；其中 82 例（89%）存在病毒感染（69 例为 EBV），1 例为脓胸，其余 9 例无相关疾病。治疗前，所有患儿均行脑脊液（CSF）评估、脑计算机断层扫描（CT）和/或磁共振成像（MRI）检查。
诊断时，43 例（47%）患儿存在 CNS 受累。12 例患儿（13%）出现神经系统症状，包括癫痫发作、共济失调、昏迷、颅神经麻痹和偏瘫。所有患儿经 8 周治疗后均有改善，但 1 例患儿后出现进行性神经系统症状，6 例患儿因进行性全身症状和/或其他原因停止治疗。15 例患儿 CSF 异常，所有患儿在治疗 6 周后均完全正常。36 例患儿（39%）有神经影像学异常；其中 5 例仍在治疗中，15 例失访，16 例完成治疗后随访。在这 16 例患儿中，12 例患儿改善，3 例患儿无变化，1 例患儿进展。在所有完成治疗后随访的 21 例 CNS 受累患儿中，10 例完全恢复，10 例改善（3 例仍有神经影像学异常），1 例临床和神经影像学均进展。
本文报道的大多数患儿患有继发性 HLH，由于 HLH 中 CNS 受累常见，建议所有 HLH 患儿在诊断时行脑 MRI 检查。治疗后 8 周内，临床和 CSF 异常常改善，但 CT/MRI 异常恢复较慢，且不常见。
