噬血细胞性淋巴组织细胞增生症患者脑脊液标本中的细胞形态学特征。
Cytomorphologic Features Found in Cerebrospinal Fluid Specimens of Hemophagocytic Lymphohistiocytosis Patients.
机构信息
Department of Pathology, University of Maryland Medical Center, Baltimore, MD, USA.
Division of Pathology, Children's National Health System, Washington, DC, USA.
出版信息
Am J Clin Pathol. 2021 Aug 4;156(3):381-390. doi: 10.1093/ajcp/aqaa248.
OBJECTIVES
Central nervous system involvement is present in 70% of patients with hemophagocytic lymphohistiocytosis (CNS-HLH). CNS-HLH is defined by neurologic deficits, neuroimaging abnormalities, or positive cerebrospinal fluid (CSF) findings. The CSF cytomorphologic spectrum of CNS-HLH, however, has not been well investigated.
METHODS
A retrospective review was performed on 64 CSF specimens from pediatric and adult patients with HLH. Ten patients had clinicoradiologic evidence of CNS involvement.
RESULTS
We identified five CSF cytomorphologic patterns: (1) hemophagocytosis, (2) vacuolated macrophages without evidence of hemophagocytosis, (3) monocytes and/or nonvacuolated macrophages, (4) acellular specimens, and (5) bloody specimens. Patterns 1 and 2 were common in CNS-HLH and rare in patients without CNS involvement. The CSF cytomorphologic patterns did not correlate well with WBC counts or protein concentration.
CONCLUSIONS
Our study offers a comprehensive view of the cytomorphologic features seen in CSF specimens from patients with HLH.
目的
噬血细胞性淋巴组织细胞增多症（HLH）患者中有 70%存在中枢神经系统受累（CNS-HLH）。CNS-HLH 定义为存在神经功能缺损、神经影像学异常或脑脊液（CSF）检查阳性结果。然而，CNS-HLH 的 CSF 细胞形态学谱尚未得到充分研究。
方法
对 64 份来自儿科和成人 HLH 患者的 CSF 标本进行回顾性分析。10 名患者具有 CNS 受累的临床影像学证据。
结果
我们确定了五种 CSF 细胞形态学模式：（1）噬血细胞现象，（2）无噬血细胞现象的空泡化巨噬细胞，（3）单核细胞和/或非空泡化巨噬细胞，（4）无细胞标本，以及（5）血性标本。模式 1 和 2 在 CNS-HLH 中常见，在无 CNS 受累的患者中罕见。CSF 细胞形态学模式与白细胞计数或蛋白浓度相关性不佳。
结论
本研究全面描述了 HLH 患者 CSF 标本的细胞形态学特征。
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