Rosai-Dorfman disease: Diagnosis and therapeutic challenges

被引：11

作者：

Aubart, F. Cohen ^{[1
,2
]}

Haroche, J. ^{[1
,2
]}

Emile, J. -F. ^{[3
]}

Charlotte, F. ^{[2
,4
]}

Barete, S. ^{[5
]}

Schleinitz, N. ^{[6
,7
]}

Donadieu, J. ^{[8
]}

Amoura, Z. ^{[1
,2
]}

机构：

[1] Hop La Pitie Salpetriere, AP HP, Ctr Natl Reference Malad Syst Rares & Histiocytos, Inst E3M,Serv Med Interne 2, F-75013 Paris, France

[2] Sorbonnes Univ, Univ Paris 06, F-75013 Paris, France

[3] Univ Versailles, Hop Ambroise Pare, AP HP, Dept Anatomopathol,EA4340, F-92104 Boulogne, France

[4] Hop La Pitie Salpetriere, AP HP, Serv Anatomopathol, F-75013 Paris, France

[5] Hop La Pitie Salpetriere, AP HP, UF Dermatol, F-75013 Paris, France

[6] Hop La Timone, Serv Med Interne, F-13385 Marseille, France

[7] Aix Marseille Univ, AP HM, F-13385 Marseille, France

[8] Hop Trousseau, AP HP, Serv Hematol, F-75012 Paris, France

来源：

REVUE DE MEDECINE INTERNE | 2018年 / 39卷 / 08期

关键词：

Rosai-Dorfman; Histiocytosis; Pachymenigitis; Lymphadenopathy; Amyloidosis; IGG4-POSITIVE PLASMA-CELLS; CENTRAL-NERVOUS-SYSTEM; MASSIVE LYMPHADENOPATHY; SINUS HISTIOCYTOSIS; EMPERIPOLESIS; METHOTREXATE; PREDNISONE; STRATEGIES; MUTATIONS; RITUXIMAB;

D O I：

10.1016/j.revmed.2018.02.011

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Rosai-Dorfman disease (RDD) was first described by the French pathologist Paul Destombes in 1965. It frequently affects children or young adults and is characterized by the presence of large histiocytes with emperipolesis. More than 50 years after this first description, the pathogenesis of this rare disease is still poorly understood. The revised classification of histiocytoses published in 2016 identified various forms of RDD, from familial RDD to IgG4-associated RDD. Almost 90% of the patients with RDD have cervical lymph nodes involvement although all the organs may virtually be involved. Outcomes are typically favorable. Treatments may be necessary in case of compression or obstruction, and are not well codified. The main therapeutic strategies rely on surgery, radiotherapy, steroids, immunosuppressive drugs or interferon-alpha and cladribine. (C) 2018 Societe Nationale Francaise de Medecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

引用

页码：635 / 640

页数：6

共 70 条

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