Department of Pathology, University of Maryland Medical Center, Baltimore, MD, USA.
Division of Pathology, Children's National Health System, Washington, DC, USA.
Am J Clin Pathol. 2021 Aug 4;156(3):381-390. doi: 10.1093/ajcp/aqaa248.
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.
A retrospective review was performed on 64 CSF specimens from pediatric and adult patients with HLH. Ten patients had clinicoradiologic evidence of CNS involvement.
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.
Our study offers a comprehensive view of the cytomorphologic features seen in CSF specimens from patients with HLH.
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。
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。
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。
本研究全面描述了 HLH 患者 CSF 标本的细胞形态学特征。
