Rastogi Anju, Jaisingh Kirti, Rajurkar Ketaki, Saran Ravindra Kumar, Singh Meeta, Baindur Siddharth, Singiri Deepthi, Gaonker Tanvi
Guru Nanak Eye Centre, Maulana Azad Medical College, New Delhi, India.
AIIMS Jodhpur, Rajasthan, India.
Eur J Ophthalmol. 2023 May;33(3):NP60-NP65. doi: 10.1177/11206721221083727. Epub 2022 Mar 1.
Rosai Dorfman disease (RDD) is a rare benign histiocytic proliferative disorder of lymph node sinuses. Langerhans cell histiocytosis (LCH) is a solitary or multisystem clonal proliferation of abnormal dendritic cells (Langerhans cells) with varied presentations. The co-occurrence of these two entities is quite rare.
A six-year-old boy presented with multiple mass lesions in the neck since two years and a nodular lesion in right upper eyelid for the past 4 months. He was diagnosed with tubercular lymphadenitis 2 years back, and was given a course of anti-tubercular therapy (ATT) elsewhere. No improvement was seen. Fine needle aspiration cytology (FNAC) of the cervical lymph nodes revealed reactive lymphadenitis while lymph node biopsy showed features of RDD. Excision biopsy of the orbital mass showed features of both RDD and LCH. The patient was started on tablet prednisolone. Six months later, complete resolution of lymph node enlargement and remaining orbital mass was noted. Post operative contrast enhanced Magnetic Resonance Imaging of head and neck was normal.
The coexistence of RDD and LCH may be a result of divergent differentiation from a common lineage or a de novo phenotypic evolution.
罗萨伊-多夫曼病（RDD）是一种罕见的淋巴结窦良性组织细胞增生性疾病。朗格汉斯细胞组织细胞增多症（LCH）是一种由异常树突状细胞（朗格汉斯细胞）引起的单发性或多系统性克隆性增殖疾病，临床表现多样。这两种疾病同时出现的情况相当罕见。
一名6岁男孩，两年来颈部出现多个肿块病变，右上眼睑出现结节性病变4个月。他于2年前被诊断为结核性淋巴结炎，并在其他地方接受了一个疗程的抗结核治疗（ATT）。未见好转。颈部淋巴结细针穿刺细胞学检查（FNAC）显示为反应性淋巴结炎，而淋巴结活检显示为RDD特征。眼眶肿块切除活检显示为RDD和LCH的特征。患者开始服用泼尼松片。6个月后，淋巴结肿大和剩余眼眶肿块完全消退。术后头颈部增强磁共振成像正常。
RDD和LCH的共存可能是由于共同谱系的分化不同或新生表型演变所致。
