European Journal of Pediatrics

, Volume 165, Issue 8, pp 536–539 | Cite as

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

  • Mercedes Bernácer-Borja
  • Miriam Blanco-Rodríguez
  • Jose Manuel Sanchez-Granados
  • Rocío Benitez-Fuentes
  • Alicia Cazorla-Jimenez
  • Carmen Rivas-Manga
Original Paper
First Online:
Received:
Accepted:

Abstract

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.

Keywords

Histiocytosis Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman 

Abbreviations

IHC

Immunohistochemical

RD

Rosai-Dorfman

SHML

Sinus histiocytosis with massive lymphadenopathy

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Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • 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. 1.Paediatric Department, Fundación Jiménez Díaz HospitalAutonomous University of MadridMadridSpain
  2. 2.Anatomía Patológica DepartmentFundación Jiménez DíazMadridSpain

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