中枢神经系统（CNS）受累在儿童非霍奇金淋巴瘤（NHL）中的特征及脑脊液流式细胞术对中枢神经系统阳性疾病的诊断价值。
Characteristics of Central Nervous System (CNS) Involvement in Children With Non-Hodgkin's Lymphoma (NHL) and the Diagnostic Value of CSF Flow Cytometry in CNS Positive Disease.
机构信息
Medical Oncology Department, Pediatric Oncology Center, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
Pathology Department, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, Beijing, China.
出版信息
Technol Cancer Res Treat. 2021 Jan-Dec;20:15330338211016372. doi: 10.1177/15330338211016372.
OBJECTIVE
To investigate the characteristics of central nervous system (CNS) involvement in children with non-Hodgkin's lymphoma (NHL) and the value of flow cytometry (FC) in the diagnosis of CNS disease in pediatric NHL.
METHODS
The data of 56 newly diagnosed pediatric NHL patients with CNS involvement (CNS+/mass, CNS+/palsy, CNS+/CSF) were analyzed. The proportions and formats of CNS disease in different pathological types were compared. In addition, FC and conventional cytology (CC) of cerebrospinal fluid (CSF) were carried out in 383 newly diagnosed NHL cases.
RESULTS
A total of 383 children with NHL were enrolled. Among these patients, 56 (14.6%) were diagnosed with positive CNS involvement (CNS+), 33 had bulky disease (tumor diameter >10 cm), 32 had bone marrow invasion, 32 had lactate dehydrogenase levels >1000 U/L, and 25 had invasion of more than 4 organs at the time of diagnosis. There were 14 patients with T lymphoblastic lymphoma (T-LBL), 9 with B lymphoblastic lymphoma (B-LBL), 26 with Burkitt's lymphoma (BL), and 2 with Epstein-Barr virus-positive diffuse large B cell lymphoma (EBV + DLBCL). Among the 56 CNS+ patients, 35 were CSF-positive (CSF+); there were 2 patients who were CSF+ via CC detection and 35 who were CSF+ via FC detection. The difference between CC and FC was statistically significant ( < 0.01). In the T-LBL group, 14 patients were CNS+/CSF, and in the B-LBL group, 8 were CNS+/mass. In the BL group, 22 patients were CNS+/mass and 15 were CNS+/CSF. In the anaplastic large-cell lymphoma group, 5 patients were CNS+/mass. Nine of the 56 CNS+ patients had events. The 2-year overall survival rate was 87% ± 0.046%, and the 2-year event-free survival rate was 76.2% ± 0.07%.
CONCLUSION
CNS+ diagnoses were more common in pediatric NHL patients with bulky disease and/or bone marrow involvement and/or involvement of more than 4 organs at the time of diagnosis, and they were also common in the EBV + DLBCL and BL groups. FC of CSF showed important clinical significance in the diagnosis of CNS disease in pediatric NHL patients, and it can be used to significantly improve the CNS+ detection rate.
目的
研究儿童非霍奇金淋巴瘤（NHL）中枢神经系统（CNS）受累的特征，以及流式细胞术（FC）在儿科 NHL 中枢神经系统疾病诊断中的价值。
方法
分析 56 例新诊断的中枢神经系统受累（CNS+/肿块、CNS+/麻痹、CNS+/CSF）的儿童 NHL 患者的数据。比较不同病理类型的中枢神经系统疾病的比例和形式。此外，对 383 例新诊断的 NHL 患者进行了脑脊液（CSF）FC 和常规细胞学（CC）检查。
结果
共纳入 383 例 NHL 患儿。其中，56 例（14.6%）诊断为中枢神经系统阳性（CNS+），33 例有肿块，32 例有骨髓侵犯，32 例乳酸脱氢酶水平>1000 U/L，25 例诊断时侵犯超过 4 个器官。14 例为 T 淋巴母细胞淋巴瘤（T-LBL），9 例为 B 淋巴母细胞淋巴瘤（B-LBL），26 例为 Burkitt 淋巴瘤（BL），2 例为 EBV 阳性弥漫大 B 细胞淋巴瘤（EBV+DLBCL）。56 例 CNS+患者中，35 例 CSF 阳性（CSF+）；2 例通过 CC 检测 CSF+，35 例通过 FC 检测 CSF+。CC 与 FC 差异有统计学意义（<0.01）。T-LBL 组 14 例为 CNS+/CSF，B-LBL 组 8 例为 CNS+/肿块。BL 组 22 例为 CNS+/肿块，15 例为 CNS+/CSF。间变大细胞淋巴瘤组 5 例为 CNS+/肿块。56 例 CNS+患者中有 9 例发生事件。2 年总生存率为 87%±0.046%，2 年无事件生存率为 76.2%±0.07%。
结论
中枢神经系统受累在有肿块和/或骨髓受累和/或诊断时侵犯超过 4 个器官的儿科 NHL 患者中更为常见，在 EBV+DLBCL 和 BL 组中也更为常见。CSF 的 FC 在儿科 NHL 患者中枢神经系统疾病的诊断中具有重要的临床意义，它可以显著提高 CNS+的检出率。
Zotero 插件