Amoako Emmanuella, Danso Kwadwo Apeadu, Akuaku Rosemary Sefakor, Ulzen-Appiah Kofi
Department of Paediatrics and Child Health, Cape Coast Teaching Hospital, Ghana University of Cape Coast, School of Medical Sciences, Cape Coast, Ghana.
Department of Paediatrics and Child Health, Cape Coast Teaching Hospital, Cape Coast, Ghana.
Case Rep Pediatr. 2022 May 26;2022:9571400. doi: 10.1155/2022/9571400. eCollection 2022.
Rosai-Dorfman disease (RDD) is a rare disease of unknown cause. It is a benign self-limiting condition characterized by the accumulation of activated histiocytes in the sinusoids of lymph nodes and/or extranodal tissues. Massive cervical lymphadenopathy as the initial manifestation tends to raise the initial odds in favour of a lymphoma, and thus reducing the threshold to performing a simple biopsy cannot be overestimated. Herein, we report a 13-year-old adolescent who presented with a progressive posterior left-sided neck swelling. Our diagnosis of RDD was established by demonstrating emperipolesis in histology and S100 positivity in immunohistochemistry as stated in the literature. Although the condition is known to be self-limiting, evidence from the literature and our case management shows that medical therapy can hasten remission in pediatric cases.
罗萨伊-多夫曼病（RDD）是一种病因不明的罕见疾病。它是一种良性自限性疾病，其特征是活化的组织细胞在淋巴结窦和/或结外组织中积聚。以颈部巨大淋巴结病为首发表现往往会增加最初诊断为淋巴瘤的可能性，因此，降低进行简单活检的阈值的重要性再怎么强调也不为过。在此，我们报告一名13岁青少年，其表现为左侧颈部后部进行性肿胀。如文献所述，我们通过组织学显示血细胞吞噬现象和免疫组化显示S100阳性来确诊RDD。尽管已知该病具有自限性，但文献证据和我们的病例管理表明，药物治疗可加速儿科病例的缓解。
