罗赛-多夫曼病1例报告

Amrita Lal Halder, Md. Abid Hossain Mollah
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摘要

Rosai Dorfman病(RDD)是一种窦性组织细胞增多症。这是一种罕见的疾病(1:200000),尤其是在儿童中,通常表现为巨大、无痛的双侧颈部淋巴结病,伴有发烧和体重减轻。白细胞增多、红细胞沉降率升高和高丙种球蛋白血症是常见的。只有对受影响的淋巴结进行组织学分析才能做出明确的诊断。以S-100蛋白和CD68抗原阳性和CD1a抗原阴性为特征的典型免疫组织化学模式是RDD的诊断。在这里,我们报告了一例RDD发生在一名10个月大的儿童身上,伴有进行性颈部淋巴结病和持续发热。淋巴结活检的组织病理学和免疫组织化学研究确定了诊断。经过密切的随访,发热和淋巴结病得到了缓解。J Bangladesh Coll Phys Surg 2022;40:299-301
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Rosai-Dorfman Disease: A Case Report
Rosai-Dorfman disease (RDD) is a type of sinus histiocytosis. It is a rare disease (1:200000), particularly in children and commonly presents with massive, painless and usually bilateral cervical lymphadenopathy along with fever and weight loss. Leukocytosis, elevated erythrocyte sedimentation rate and hypergammaglobulinemia are common. A definitive diagnosis can only be made by histological analysis of affected lymph nodes. Emperipolesis and a typical immunohistochemical pattern characterized by positivity for S-100 protein and CD68 antigen and negativity for CD1a antigen are diagnostic for RDD. Here we report a case of RDD occurring in a 10-month-old child with progressive cervical lymphadenopathy and persistent fever. Histopathological and immunohistochemistry studies of a lymph node biopsy established the diagnosis. A watchful follow-up resulted in the resolution of fever and lymphadenopathy. J Bangladesh Coll Phys Surg 2022; 40: 299-301
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