Abstract:
Rosai-Dorfman Disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is an uncommon histiocytic condition characterized by massive histopathological aggregation of CD1-a negative, CD68-positive, and S100-positive histiocytes. It was initially described by Destombes in 1965 under the term \"adenitis with lipid excess.\" However, it is named after Rosai and Dorfman who reported further histopathological features of the disease in 1969. The diagnosis of this non-Langerhans cell histiocytosis can be challenging and requires high clinical suspicion. The diagnostic process usually involves imaging, tissue biopsies, and genetic testing as needed. In this case series, we are presenting three cases of rare disease. Case 2 had both nodal and extranodal forms, which makes this case rarer than cases 1 and 3, which present with extranodal lesions.
摘要:
Rosai-Dorfman病(RDD)也被称为窦组织细胞增生症伴大量淋巴结病,是一种罕见的组织细胞疾病,其特征是CD1-a阴性的大量组织病理学聚集,CD68阳性,和S100阳性组织细胞。它最初由Destombes于1965年在术语“脂质过量的腺炎”下描述。\"然而,它以Rosai和Dorfman的名字命名,他们在1969年报告了该疾病的进一步组织病理学特征。这种非朗格汉斯细胞组织细胞增生症的诊断可能具有挑战性,需要高度的临床怀疑。诊断过程通常涉及成像,组织活检,根据需要进行基因检测。在这个系列中,我们提出了三例罕见疾病。病例2有结节和结外两种形式,这使得这种情况比病例1和3更罕见,后者存在结外病变。