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Sinus histiocytosis with massive lymphadenopathy

Topic Completed: 1 July 2014

Minor changes: 1 March 2019

Copyright: 2003-2019,, Inc.

PubMed Search: Sinus histiocytosis with massive lymphadenopathy pleural

Vaidehi Avadhani, M.D.
Page views in 2019: 169
Page views in 2020 to date: 131
Cite this page: Avadhani V. Sinus histiocytosis with massive lymphadenopathy. website. Accessed May 29th, 2020.
  • Also called Rosai-Dorfman disease (RDD)
  • Predominantly occurs in first and second decades of life
  • Greater incidence in blacks
  • Most common in lymph nodes, head and neck; also upper respiratory tract, skin, skeletal system, CNS
  • Almost every organ has been described as a site of disease
  • Very rare in pleura (one case report to date)
  • Etiology unknown but its histiocytes are not clonal
  • Some studies have indicated it belongs to a different spectrum of disease than IgG4 disease, others have questioned if they are associated (Histopathology 2014;64:455)
  • Association with autoimmune lymphoproliferative syndrome has also been reported
  • No evidence of association with EBV, HHV6
Clinical features
  • Fever, leukocytosis, elevated ESD, polyclonal hypergammaglobulinemia are most common
  • Extranodal RDD may spare lymph nodes
Radiology images

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Lytic lesion over scaphoid bone

Case reports
  • May undergo spontaneous resolution or follow a protracted clinical course
  • Chemotherapy has proven effective in some cases at other sites
Clinical images

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Swelling over left wrist

Microscopic (histologic) description
  • Diffuse infiltration by histiocytes, lymphocytes and occasional neutrophils
  • Histiocytes have abundant clear cytoplasm (due to lipids) or eosinophilic cytoplasm and a large vesicular nucleus
  • "Emperipolesis" or lymphophagocytosis is common: intact RBCs, lymphocytes or plasma cells in cytoplasm but this is not pathognomonic
  • Fibrosis is common
  • Increased in plasma cells has also been described
  • Extranodal disease has more fibrosis and less lymphophagocytosis
Microscopic (histologic) images

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Lymph node architecture preserved

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Histiocytes with emperipolesis

Cytology images

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Large histiocytes

Positive stains
Negative stains
Molecular / cytogenetics description
  • No evidence of clonality
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