Alexis Elira Dokékias, Firmine Olivia Galiba Atipo Tsiba, Fortuné Bissiko, Antoine Martin
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引用次数: 0
Abstract
A 8-year-old schoolgirl from West Africa with no previous pathological history was admitted to the haematology department of the Brazzaville University Hospital for the management of cervical adenopathy. The diagnosis of sinus histiocytosis or Destombes-Rosaï-Dorfman disease was retained and the patient was treated with PO corticosteroids (methylprednisolone 32 mg/d then 16 mg/d). Given the rarity and uncertain aetiopathogeny of this syndrome, treatment is poorly codified. It includes corticosteroid therapy, immunomodulators and sometimes chemotherapy, radiotherapy or surgery, indicated in case of clinical manifestations of local organ compression. The disease may regress spontaneously. Its benignity does not justify systematic treatment in the absence of complications.
布拉柴维尔大学医院(Brazzaville University Hospital)血液科收治了一名来自西非的 8 岁女学生,她以前没有病史。患者被诊断为窦组织细胞增生症或Destombes-Rosaï-Dorfman病,并接受了PO皮质类固醇治疗(甲基强的松龙32毫克/天,然后是16毫克/天)。由于这种综合征十分罕见,病因也不确定,因此治疗方法也不十分规范。治疗方法包括皮质类固醇治疗、免疫调节剂,有时还包括化疗、放疗或手术(在出现局部器官受压的临床表现时)。这种疾病可能会自发消退。在没有并发症的情况下,其良性程度并不证明有必要进行系统治疗。