Systemic Rosai-Dorfman-Destombes disease associated with a myelodysplastic syndrome. Efficacy of hematologic treatment

被引：0

作者：

Deshayes, R. ^{[1
]}

Eustache, M. ^{[1
]}

Moreau, A. ^{[2
]}

Peterlin, P. ^{[3
]}

Neel, A. ^{[1
]}

Masseau, A. ^{[1
]}

Bodet-Milin, C. ^{[4
]}

Hamidou, M. ^{[1
]}

机构：

[1] Hop Hotel Dieu, Serv Med Interne, F-44000 Nantes, France

[2] Hop Hotel Dieu, Lab Anatomopathol, F-44000 Nantes, France

[3] Hop Hotel Dieu, Serv Hematol, F-44000 Nantes, France

[4] Univ Nantes, Serv Med Nucl, CHU Hotel Dieu Nantes, CRCINA,Inserm,CNRS,Univ dAngers, F-44000 Nantes, France

来源：

REVUE DE MEDECINE INTERNE | 2021年 / 42卷 / 04期

关键词：

Destombes-Rosai-Dorfman; histiocytosis; myelodysplastic syndrome; azacytidine; LANGERHANS CELL HISTIOCYTOSIS; ERDHEIM-CHESTER DISEASE; SINUS HISTIOCYTOSIS; AUTOIMMUNE; NEOPLASMS;

D O I：

10.1016/j.revmed.2021.01.001

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Introduction. - 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. Case report. - 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. Conclusion. - Our observation discusses the interest of the treatment of myelodysplastic syndrome for the management of associated extra-hematological manifestations. (C) 2021 Societe Nationale Francaise de Medecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

引用

页码：281 / 284

页数：4

共 31 条

[31] Phenotypic intrafamilial variability including H syndrome and Rosai-Dorfman disease associated with the same c.1088G>A mutation in the SLC29A3 gene
Chouk, Hamza
Ben Rejeb, Mohamed
Boussofara, Lobna
Elmabrouk, Haifa
Ghariani, Najet
Sriha, Badreddine
Saad, Ali
H'Mida, Dorra
Denguezli, Mohamed
HUMAN GENOMICS, 2021, 15 (01)

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