Dalia Samir, Sagatys Elizabeth, Sokol Lubomir, Kubal Timothy
Mercy Clinic Oncology-Hematology, Joplin, MO 64804, USA.
Cancer Control. 2014 Oct;21(4):322-7. doi: 10.1177/107327481402100408.
Rosai-Dorfman disease (RDD) is a rare, nonmalignant clinical entity characterized by a group of clinical symptoms and characteristic pathological features.
Articles that reviewed tumor biology, clinical features, pathology, and treatment for RDD were identified in a search of the literature for the years 1990 to 2014. The results from this body of literature were reviewed and summarized.
Patients with RDD generally present with massive, painless cervical lymphadenopathy, fevers, and elevated inflammatory markers. Extranodal disease is typical, with the most common sites being the skin and the central nervous system. Rarely, the gastrointestinal tract is involved. Immunohistochemistry remains the mainstay of diagnosis with S100 and CD68 positive cells while CD1a will be negative of involved histiocytes. Histologically, the disease shows the classical characteristic finding of emperipolesis. Many patients do not require treatment; however, surgical resection remains the mainstay of treatment for symptomatic disease. The role of steroids, chemotherapy, and radiation therapy continue to be based on small case series and case reports.
RDD has a variable clinical presentation; therefore, a high degree of suspicion and a thorough pathological review are necessary to diagnose this rare clinical entity. Although some patients will experience spontaneous resolution, others may require surgical resection or steroid therapy and radiation or chemotherapy. Given the rarity of the disease and the lack of a clear therapeutic pathway, referring patients to a tertiary center is recommended for confirming the diagnosis and treatment considerations.
罗萨伊-多夫曼病（RDD）是一种罕见的非恶性临床病症，其特征为一组临床症状和特征性病理表现。
在检索1990年至2014年的文献时，确定了回顾RDD肿瘤生物学、临床特征、病理学和治疗的文章。对这一系列文献的结果进行了回顾和总结。
RDD患者通常表现为颈部无痛性巨大淋巴结肿大、发热及炎症标志物升高。结外病变很典型，最常见的部位是皮肤和中枢神经系统。胃肠道受累罕见。免疫组化仍是诊断的主要手段，S100和CD68阳性细胞，而受累组织细胞的CD1a呈阴性。组织学上，该病表现出经典的嗜血细胞现象特征性表现。许多患者无需治疗；然而，手术切除仍是有症状疾病的主要治疗方法。类固醇、化疗和放疗的作用仍基于小病例系列和病例报告。
RDD临床表现多样；因此，高度怀疑并进行全面的病理检查对于诊断这种罕见的临床病症很有必要。虽然一些患者会自行缓解，但其他患者可能需要手术切除或类固醇治疗以及放疗或化疗。鉴于该病罕见且缺乏明确的治疗途径，建议将患者转诊至三级中心以确诊并考虑治疗。
