Magableh Hamzah M, Jaber Hamzh D, Magableh Ahmad M, Alrabiah Mohammed A, Dahhan Abdulaziz F, Azzam Ayman Z, Amin Tarek
College of Medicine, Alfaisal University, Riyadh, SAU.
Department of Surgical Oncology, Oncology Center, King Faisal Specialist Hospital and Research Center, Riyadh, SAU.
Cureus. 2023 Feb 19;15(2):e35193. doi: 10.7759/cureus.35193. eCollection 2023 Feb.
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.
罗萨伊-多夫曼病(RDD),又称伴巨大淋巴结病的窦性组织细胞增生症,是一种罕见的组织细胞病症,其特征为CD1-a阴性、CD68阳性和S100阳性组织细胞的大量组织病理学聚集。它最初由德斯顿贝斯于1965年以“脂质过多性腺炎”这一术语描述。然而,它是以罗萨伊和多夫曼命名的,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。诊断过程通常包括影像学检查、组织活检以及必要时的基因检测。在这个病例系列中,我们展示了三例罕见疾病。病例2同时具有淋巴结和结外形式,这使得该病例比病例1和病例3更罕见,病例1和病例3表现为结外病变。