关键词: benign lymphoproliferative hemophagocytic lympho-histiocytosis intracranial neoplasms neuro radiology pet ct scan rosai-dorfman disease sinus histiocytosis with massive lymphadenopathy

来  源:   DOI:10.7759/cureus.32605   PDF(Pubmed)

Abstract:
Sinus histiocytosis with massive lymphadenopathy (SHML), an alternative term for Rosai-Dorfman disease (RDD), is a rare benign idiopathic immune-related lymphoproliferative condition. The central nervous system (CNS) has been documented to be involved in RDD, although lymph nodes are the organs that are most frequently and primarily associated with the disease manifestation. Nonetheless, CNS involvement in RDD is rare and poorly understood. As a result, there is a lack of a solid basis for therapeutic approaches for CNS involvement in RDD. Here, we present a case of RDD with cerebral involvement, a rare presentation of RDD with atypical symptoms. A brief assessment of the radiographic appearance, histological findings, and the peculiar manifestations of the disease is provided.
摘要:
窦组织细胞增生症伴巨大淋巴结肿大(SHML),Rosai-Dorfman病(RDD)的替代术语,是一种罕见的良性特发性免疫相关淋巴增生性疾病。中枢神经系统(CNS)已被证明与RDD有关,尽管淋巴结是最常见的器官,主要与疾病表现有关。尽管如此,中枢神经系统参与RDD是罕见的和知之甚少。因此,中枢神经系统参与RDD的治疗方法缺乏坚实的基础.这里,我们提出了一个脑受累的RDD病例,罕见的RDD表现为非典型症状。对射线照相外观的简要评估,组织学发现,并提供了这种疾病的特殊表现。
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