噬血综合征又称噬血细胞性淋巴组织细胞增生症(HLH)属于非肿瘤性组织细胞增生性疾病,是由原发性或者继发性免疫异常所致,常见于儿童,发病急骤,进展迅速,常引起多个器官严重受损;其中,中枢神经系统(Central Nervous System, CNS)受累可达63%~93%,且已有越来越多的研究显示:CNS受累是HLH预后不良的独立危险因素,儿童CNS受累的死亡率可高达75%。临床上,明确诊断HLH后,一旦出现神经系统症状、脑脊液或头颅影像学其中一种异常时,CNS受累才能诊断。其中,MRI检查因其无创、无辐射的优势已成为HLH患儿CNS受累的常规评估手段;出现CNS受累时,患者在MRI阳性表现率较高,可有助于早期识别病情。所以,该文主要针对于HLH-CNS受累的认识及研究情况,尤其是HLH-CNS受累的MRI相关影像学研究进展方面进行阐述。Hemophagocytic syndrome, also known as hemophagocytic lymphohistiocytosis (HLH), is a non-neoplastic disorder characterized by the proliferation of tissue macrophages, which can result from primary or secondary immune abnormalities and is most commonly seen in children. The onset of the disease is acute, and it progresses rapidly, often leading to severe damage in multiple organs. Research indicates that central nervous system (CNS) involvement occurs in as many as 63%~93% of cases. Increasing evidence demonstrates that CNS involvement is an independent risk factor for poor prognosis in HLH, with mortality rates among affected children reaching up to 75%. In clinical practice, diagnosing CNS involvement relies on a confirmed HLH diagnosis;it can be established when any of the following are present: CNS symptoms, cerebrospinal fluid abnormalities, or cranial imaging abnormalities. MRI has become the standard assessment tool for evaluating CNS involvement in HLH patients due to its non-invasive and radiation-free advantages. When CNS involvement is present, patients typically exhibit a higher rate of positive findings on MRI, aiding in early identification of the condition. This article will focus on the understanding and research status of HLH and its CNS involvement, particularly emphasizing advancements in MRI imaging studies related to HLH-CNS involvement.
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