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罗萨伊-多夫曼病表现为一名巴勒斯坦男童的自身免疫性溶血性贫血:病例报告

Rosai-Dorfman disease presenting as autoimmune hemolytic anemia in a male child in Palestine: a case report.

作者信息

Njoum Yumna, Abu-Hilal Lila H, Jabbarin Farah, Bannoura Sami, Mtour Sameer, Itmaizeh Muaath

机构信息

Faculty of Medicine, Al-Quds University, Jerusalem, Palestine.

Department of Cardiology, Al-Makassed Hospital, Jerusalem, Palestine.

出版信息

Oxf Med Case Reports. 2023 Jul 18;2023(7):omad078. doi: 10.1093/omcr/omad078. eCollection 2023 Jul.

Abstract

Rosai-Dorfman disease (RDD) is a rare, benign non-Langerhans cell histiocytosis predominantly affects lymph nodes and skin. Despite its benign nature, RDD can cause serious hematological complications. A 14-year-old male, presented with 3-month history of hemolytic anemia, lymphadenopathy, hepatosplenomegaly and rash. After thorough investigation, RDD was diagnosed by mediastinal lymph node biopsy that revealed presence of S100 and CD68-positive cells with absence of CD1a confirming the diagnosis of RDD. Treatment involved combination of steroids and Rituximab, which proved to be highly effective. The patient had dramatic improvement and entered remission, with follow-up period of 2 years. It is important to note that although RDD is a rare disease, it causes severe complications, as evidenced by the patient's parameters. Thus, prompt diagnosis and treatment are paramount. Histological diagnosis is of great value, as it helps confirming and guiding treatment decisions. With the right treatment, patients can experience great recovery and quality of life.

摘要

罗萨伊-多夫曼病(RDD)是一种罕见的良性非朗格汉斯细胞组织细胞增多症,主要累及淋巴结和皮肤。尽管其本质为良性,但RDD可导致严重的血液学并发症。一名14岁男性,有3个月的溶血性贫血、淋巴结病、肝脾肿大和皮疹病史。经过全面检查,通过纵隔淋巴结活检诊断为RDD,活检显示存在S100和CD68阳性细胞,而CD1a阴性,从而确诊为RDD。治疗采用类固醇和利妥昔单抗联合使用,结果证明非常有效。患者病情显著改善并进入缓解期,随访期为2年。需要注意的是,尽管RDD是一种罕见疾病,但正如患者的各项指标所示,它会引起严重并发症。因此,及时诊断和治疗至关重要。组织学诊断具有重要价值,因为它有助于确诊并指导治疗决策。通过正确的治疗,患者可以实现良好的康复并提高生活质量。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4258/10359060/250e76af458a/omad078f1.jpg

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