Kitticharoenjit Ponrachet, Supakul Nucharin, Rujkijyanont Piya, Traivaree Chanchai, Photia Apichat, Monsereenusorn Chalinee
Department of Pediatrics, Phramongkutklao Hospital, Bangkok 10400, Thailand.
Department of Radiology and Imaging Science, Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, IN 46202, United States of America.
Asian Biomed (Res Rev News). 2021 Aug 20;15(4):171-181. doi: 10.2478/abm-2021-0022. eCollection 2021 Aug.
Langerhans cell histiocytosis (LCH) is a rare disease characterized by the various systems involved and clinical manifestations with a wide range of symptoms.
To describe clinical characteristics, imaging, treatment, and outcomes of pediatric LCH at Phramongkutklao Hospital, Bangkok, Thailand.
We conducted a 20-year retrospective review of the medical records of patients diagnosed with LCH from birth to 21 years old from January 1, 1997, to December 31, 2016.
In all, 14 patients with median age of 2.5 years were studied. Six (43%) patients had single-system (SS) LCH. Five patients (63%) with multisystem (MS) LCH (n = 8. 57%) had risk-organ involvement (RO+). All patients had plain X-ray imaging of their skull with 11 (79%) showing abnormal findings. Tc-99m bone imaging and fluorodeoxyglucose F18 (FDG) positron emission tomography (PET)-computed tomography (CT) demonstrated abnormal findings in 8 (89%) and 4 (29%) patients, respectively. The 5-year event-free survival (EFS) for patients with RO+ MS-LCH was less than that for those without risk-organ involvement (RO-) MS-LCH and SS-LCH (20% vs. 100%, = 0.005). Hematological dysfunction, hypoalbuminemia, and conjugated hyperbilirubinemia may be worse prognostic factors for RO+ MS-LCH.
FDG-PET-CT might have a greater accuracy to detect LCH disease than conventional plain X-ray and Tc-99m bone imaging. RO+ MS-LCH has been encountered with relapse and poor outcomes. Hematopoietic involvement, hypoalbuminemia, and conjugated hyperbilirubinemia may be worse prognostic factors for RO+ MS-LCH.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见疾病，其特征是累及多个系统且临床表现多样、症状广泛。
描述泰国曼谷诗里拉吉医院儿科LCH的临床特征、影像学表现、治疗及预后。
我们对1997年1月1日至2016年12月31日期间诊断为LCH的从出生至21岁患者的病历进行了为期20年的回顾性研究。
共研究了14例患者，中位年龄为2.5岁。6例（43%）患者为单系统（SS）LCH。5例（63%）多系统（MS）LCH患者（n = 8，57%）存在风险器官受累（RO+）。所有患者均进行了颅骨平片检查，11例（79%）显示异常结果。锝-99m骨显像和氟脱氧葡萄糖F18（FDG）正电子发射断层扫描（PET）-计算机断层扫描（CT）分别在8例（89%）和4例（29%）患者中显示异常结果。RO+ MS-LCH患者的5年无事件生存率低于无风险器官受累（RO-）的MS-LCH和SS-LCH患者（20%对100%，P = 0.005）。血液系统功能障碍、低白蛋白血症和结合胆红素血症可能是RO+ MS-LCH预后较差的因素。
FDG-PET-CT在检测LCH疾病方面可能比传统的平片和锝-99m骨显像具有更高的准确性。RO+ MS-LCH易复发且预后不良。造血系统受累、低白蛋白血症和结合胆红素血症可能是RO+ MS-LCH预后较差的因素。
