Maia R C, de Meis E, Romano S, Dobbin J A, Klumb C E
Programa de Pesquisa em Hemato-Oncologia Molecular, Instituto Nacional de Câncer, Rio de Janeiro, RJ, Brasil.
Divisão Clínica, Hospital do Câncer-I, Instituto Nacional de Câncer, Rio de Janeiro, RJ, Brasil.
Braz J Med Biol Res. 2015 Jan;48(1):6-12. doi: 10.1590/1414-431X20144110. Epub 2014 Oct 10.
Rosai-Dorfman disease (RDD) is a nonmalignant histiocytic disorder of unknown origin that is extremely rare. By immunohistochemistry, the RDD cells are characteristically S-100 positive and CD1a negative. Emperipolesis is a common histopathological finding, although not specific for RDD. Lymph node and cutaneous manifestations are most frequent, but diverse organs can be affected. The clinical course is unpredictable regardless of treatment. Here, we present a series of 8 cases presenting lymph node and/or cutaneous lesions. Lymph node involvement was seen in diverse regions, including mediastinal and retroperitoneal. The treatment response to steroids was diversified, and the chemotherapy response was disappointing. Associated autoimmune diseases (Sjögren syndrome and antiphospholipid syndrome) were observed in 2 patients. Regardless of therapy modality, these patients exhibited a favorable prognosis in a follow-up duration that ranged from 15 to 80 months.
罗萨伊-多夫曼病（RDD）是一种起源不明的非恶性组织细胞疾病，极为罕见。通过免疫组织化学检查，RDD细胞的特征是S-100阳性而CD1a阴性。嗜组织细胞现象是一种常见的组织病理学表现，尽管并非RDD所特有。淋巴结和皮肤表现最为常见，但不同器官均可受累。无论治疗如何，临床病程都不可预测。在此，我们报告了一系列8例出现淋巴结和/或皮肤病变的病例。在包括纵隔和腹膜后等不同区域均可见淋巴结受累。对类固醇的治疗反应各异，化疗反应令人失望。2例患者观察到相关的自身免疫性疾病（干燥综合征和抗磷脂综合征）。无论采用何种治疗方式，这些患者在15至80个月的随访期内均表现出良好的预后。
