Lymph nodes & spleen, nonlymphoma
Lymph nodes-inflammatory / reactive disorders
Sinus histiocytosis with massive lymphadenopathy

Topic Completed: 1 June 2006

Minor changes: 10 September 2020

Copyright: 2003-2021,, Inc.

PubMed Search: Sinus histiocytosis with massive lymphadenopathy[TI] lymph node

Nat Pernick, M.D.
Page views in 2020: 12,490
Page views in 2021 to date: 3,194
Cite this page: Pernick N. Sinus histiocytosis with massive lymphadenopathy. website. Accessed March 4th, 2021.
Definition / general
  • Massive painless bilateral lymph node enlargement in neck, of unknown etiology
  • Rare; 25% involve extranodal sites (skin, upper respiratory tract, bone) but massive lymphadenopathy is usually also present
  • Also called Rosai-Dorfman disease
Clinical features
  • Associated with fever, leukocytosis, anemia, elevated sedimentation rate, polyclonal hypergammaglobulinemia
  • Usually age 20 years or less but can affect any age
  • Initial cases from South Africa; now from U.S., Western Europe, Africa and Caribbean
  • May spontaneously resolve; extranodal cases may persist for decades
  • May cause death due to amyloidosis, involvement of a vital organ or immunologic abnormalities
  • May coexist with lymphoma in same node (Mod Pathol 2000;13:414)
Radiology images

AFIP images

Disease in bone

Case reports
Lymph nodes:
  • None in most cases
Clinical images

AFIP images

Skin lesion

Gross images

Contributed by Dr. Semir Vranic

Thigh lesion

Microscopic (histologic) description
    Lymph node:
  • Distinctive features are emperipolesis, plasma cells, distention of nodal sinuses
  • Capsular and pericapsular inflammation and fibrosis
  • Dilated sinuses filled with large histiocytes containing intact lymphocytes (emperipolesis, lymphocytophagocytosis) and with extremely large, round, vesicular nuclei with a delicate nuclear membrane and prominent nucleoli
  • Histiocytes may exhibit atypia and be multinucleated
  • Polyclonal plasma cells and lymphocytes
  • Plasma cells frequently surround prominent high endothelial venules
  • Remaining node contains plasma cells with Russell bodies
  • No / rare internodal fibrosis
Microscopic (histologic) images

AFIP images

Lymph node: H&E and S100

Lymph node


Extranodal: H&E and S100


Case #302

45 year old woman with breast mass

Contributed by Dr. Semir Vranic

Subcutaneous right thigh



Cytology description
  • Histiocytes with large nuclei and emperipolesis
  • Lymphocytes are within cytoplasmic vacuoles
  • Histiocytes may have atypia; also reactive lymphocytes (Diagn Cytopathol 2001;24:42)
Cytology images

AFIP images

Touch imprint of lymph node

Positive stains
Negative stains
Molecular / cytogenetics description
  • Not clonal
Differential diagnosis
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