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Deshayes, R.; Eustache, M.; Moreau, A.; Péterlin, P.; Néel, A.; Masseau, A.; Bodet-Milin, C.; Hamidou, M.
La revue de medecine interne, April 2021, 2021-04-00, 2021-04, Volume: 42, Issue: 4Journal Article
Nous rapportons une observation originale de maladie de Destombes-Rosai-Dorfman multifocale et réfractaire, associée à un syndrome myélodysplasique. Le traitement de la myélodysplasie a permis une très bonne réponse prolongée des 2 pathologies. Un patient de 35 ans était exploré pour une exophtalmie bilatérale, rapportée histologiquement à une maladie de maladie de Destombes-Rosai-Dorfman. Le bilan d’extension retrouvait une atteinte sinusienne, rénale et ganglionnaire. Il était traité successivement, sans succès, par corticoïdes, colchicine, méthotrexate, infliximab, cladribine et tociluzimab. L’apparition secondaire d’une myélodysplasie (AREB score IPSS intermédiaire-2) faisait proposer un traitement d’induction par aracytine et idarubicine, avec entretien par azacytidine pendant 2 ans. Avec un recul de 5 ans, le patient est en rémission du syndrome myélodysplasique et de la maladie de Destombes-Rosai-Dorfman. Notre observation discute l’intérêt du traitement du syndrome myélodysplasique, dans la prise en charge des manifestations extra-hématologiques associées. 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.
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