Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): clinico-pathological study of three cases

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• 作者：Mercedes Bernácer-Borja (1)
  Miriam Blanco-Rodríguez (1)
  Jose Manuel Sanchez-Granados (1)
  Rocío Benitez-Fuentes (1)
  Alicia Cazorla-Jimenez (2)
  Carmen Rivas-Manga (1)
  
• 关键词：Histiocytosis ; Sinus histiocytosis with massive lymphadenopathy ; Rosai ; Dorfman
• 刊名：European Journal of Pediatrics
• 出版年：2006
• 出版时间：August 2006
• 年：2006
• 卷：165
• 期：8
• 页码：536-539
• 全文大小：206KB
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  2. Drosos AA, Georgiadis AN, Metafratzi ZM, Voulgari PV, Efremidis SC, Bai M (2004) Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with primary Sj?gren’s syndrome. Scand J Rheumatol 33:119-22 CrossRef
  3. Eisen RN, Buckley PJ, Rosai J (1990) Immunophenotypic characterization of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Semin Diagn Pathol 7:74-2
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  5. Foucar E, Rosai J, Dorfman RF (1990) Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 7:19-3
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  7. Harley EH (1991) Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) in a patient with elevated Epstein-Barr virus titers. J Natl Med Assoc 83:922-24
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  11. Oliva H, Rosai J, C Rivas, JA Abad (1978) Sinus Histiocytosis with massive lymphadenopathy. A Light electron microscopy and immunocytochemical study with antigenic stimulation. Morf Norma Patol A 2:609-20
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  14. Rivas C, Oliva H, Martín C, Moragas A, Sarasa JL, Abad JA, Calderín F, Villalobos E, Boixadós JR, Castro A, Benítez J (1985) Rosai-Dorfman Disease. A clinico-morphological and immunocytochemical study of 8 cases. Patología 18:213-30
  15. Rodriguez-Galindo C, Helton KJ, Sanchez ND, Rieman M, Jeng M, Wang W (2004) Extranodal Rosai-Dorfman disease in children. J Pediatr Oncol 26:19-4 CrossRef
  16. Rosai J, Dorfman RF (1969) Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinico-pathological entity. Arch Pathol 87:63-0
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• 作者单位：Mercedes Bernácer-Borja (1)
  Miriam Blanco-Rodríguez (1)
  Jose Manuel Sanchez-Granados (1)
  Rocío Benitez-Fuentes (1)
  Alicia Cazorla-Jimenez (2)
  Carmen Rivas-Manga (1)
  
  1. Paediatric Department, Fundación Jiménez Díaz Hospital, Autonomous University of Madrid, Madrid, Spain
  2. Anatomía Patológica Department, Fundación Jiménez Díaz, Avenida Reyes Católicos, 2, 28040, Madrid, Spain
  

文摘

Introduction We report three cases of sinus histiocytosis, a rare disease of unknown aetiology with massive lymphadenopathy (SHML), also known as Rosai-Dorfman (RD) disease, in a paediatric population. This proliferative histiocytic disorder is defined by histological and immunohistochemical (IHC) characteristics and can manifest as nodal involvement with variable enlargement of the lymph nodes (two cases) and extranodal manifestations involving skin and larynx involvement (one case). One patient had hypergammaglobulinemia. The morphological investigation revealed that all lymph nodes showed hyperplasia of sinuses with abundant histiocytic cell with intracytoplasmic lymphocytes. Skin and larynx biopsies showed a histiocyte and lymphocyte infiltrate with similar characteristics. An ultrastructural study was carried out on material from one patient. In the IHC study, SHML cells expressed phagocytic markers such as CD68 and S100, but markers for Langerhan’s (CD1a) or dendritic cells (DRC, CD23 and CNA42) were absent. Two patients had a complete remission after surgical excision and no other treatment, but the third patient was treated with radiotherapy after a relapse with obstruction of the upper airway. Conclusion This disorder must be considered in the differential diagnosis of young patients who exhibit massive or multiple lymphadenopathies, especially when involvement of the cervical area occurs. Due to the good outcome of the disease, a conservative approach is justified.