Laboratory of Hematologic Diseases, Beijing Pediatric Research Institute, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics, Capital Medical University, Key Laboratory of Major Diseases in Children, Ministry of Education, Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Int J Lab Hematol. 2020 Dec;42(6):788-795. doi: 10.1111/ijlh.13304. Epub 2020 Aug 26.
Langerhans cell histiocytosis (LCH) is a rare disease caused by clonal proliferation of CD1a CD207 cells. Distinguishing pituitary involvement was essential in stratification and treatment of patients with LCH. The diagnosis of pituitary involvement is mainly dependent on hormone abnormalities in the anterior pituitary and magnetic resonance imaging (MRI) scanning in posterior pituitary. Diabetes insipidus (DI) is a serious sequelae and often occurred with pituitary involvement. It is reported that osteopontin (OPN) is highly secreted in the cerebrospinal fluid (CSF) of patients with neurodegenerative diseases in LCH (LCH-ND). However, patients with posterior pituitary involvement account for a larger portion in our hospital. Whether the OPN level could be an auxiliary diagnostic marker for the posterior pituitary involvement or not is still unknown.
In our study, we collected CSF samples of 57 children with LCH. The secreted OPN (sOPN) levels in CSF were measured through enzyme-linked immunosorbent assay (ELISA).
After the retrospective analysis of 57 patients with LCH, we found that the sOPN levels in CSF of children with posterior pituitary involvement were significantly higher than that of other groups. After the Pearson Chi-Square test, Fisher's exact test and ROC analysis, we found that the sOPN levels were significantly correlated with posterior pituitary involvement. The cut-off value is 214.14 ng/mL.
The sOPN levels were elevated in CSF of LCH children with posterior pituitary involvement. Analysis of the sOPN level may provide more accurate auxiliary diagnostic techniques for the clinic.
朗格汉斯细胞组织细胞增生症（LCH）是一种由 CD1a CD207 细胞克隆性增殖引起的罕见疾病。区分垂体受累对于 LCH 患者的分层和治疗至关重要。垂体受累的诊断主要依赖于垂体前叶激素异常和垂体后叶磁共振成像（MRI）扫描。尿崩症（DI）是一种严重的后遗症，常与垂体受累有关。据报道，骨桥蛋白（OPN）在 LCH（LCH-ND）患者的神经退行性疾病的脑脊液（CSF）中高度分泌。然而，在我院，后叶垂体受累的患者占较大比例。OPN 水平是否可以作为后叶垂体受累的辅助诊断标志物尚不清楚。
在本研究中，我们收集了 57 例 LCH 患儿的脑脊液样本。通过酶联免疫吸附试验（ELISA）测量脑脊液中分泌的 OPN（sOPN）水平。
对 57 例 LCH 患儿进行回顾性分析后，我们发现后叶垂体受累患儿的 CSF 中 sOPN 水平明显高于其他组。经过 Pearson Chi-Square 检验、Fisher 确切检验和 ROC 分析，我们发现 sOPN 水平与后叶垂体受累显著相关。截断值为 214.14 ng/mL。
LCH 患儿的 CSF 中 sOPN 水平升高。分析 sOPN 水平可能为临床提供更准确的辅助诊断技术。
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