Deshayes R, Eustache M, Moreau A, Péterlin P, Néel A, Masseau A, Bodet-Milin C, Hamidou M
Service de médecine interne, Hôtel-Dieu, Nantes 44000, France.
Service de médecine interne, Hôtel-Dieu, Nantes 44000, France.
Rev Med Interne. 2021 Apr;42(4):281-284. doi: 10.1016/j.revmed.2021.01.001. Epub 2021 Jan 21.
We report an original observation of multifocal refractory Destombes-Rosai-Dorfman disease associated with a myelodysplastic syndrome. The treatment of myelodysplasia allowed a good and prolonged response of both pathologies.
A 35-year-old patient was investigated for bilateral exophthalmia, histologically related to Destombes-Rosai-Dorfman disease. The extension workup showed sinus, kidney and lymph node involvement. It was treated unsuccessfully with corticosteroids, colchicine, methotrexate, infliximab, cladribine and tociluzimab. The secondary appearance of myelodysplasia (AREB IPSS score intermediate-2) led to induction treatment with aracytin and idarubicin, and maintenance with azacytidine for 2 years. With 5 years of follow-up, the patient is in remission both of the myelodysplastic syndrome and Destombes-Rosai-Dorfman disease.
Our observation discusses the interest of the treatment of myelodysplastic syndrome for the management of associated extra-hematological manifestations.
我们报告了一例与骨髓增生异常综合征相关的多灶性难治性德斯顿布-罗萨伊-多夫曼病的原始观察病例。骨髓增生异常综合征的治疗使两种病症均获得了良好且持久的缓解。
一名35岁患者因双侧眼球突出接受检查，组织学检查显示与德斯顿布-罗萨伊-多夫曼病相关。进一步检查发现鼻窦、肾脏和淋巴结受累。患者接受了皮质类固醇、秋水仙碱、甲氨蝶呤、英夫利昔单抗、克拉屈滨和托珠单抗治疗，但均未成功。骨髓增生异常综合征（修订国际预后评分系统[IPSS-R]评分为中-2）的继发出现导致患者接受了阿糖胞苷和伊达比星诱导治疗，并使用阿扎胞苷维持治疗2年。经过5年的随访，患者的骨髓增生异常综合征和德斯顿布-罗萨伊-多夫曼病均处于缓解状态。
我们的观察探讨了治疗骨髓增生异常综合征对管理相关血液外表现的意义。
