Rosai-Dorfman disease

Rosai-Dorfman disease, abbreviated RDD, is a rare lymph node pathology.

Rosai-Dorfman disease
Diagnosis in short
Rosai-Dorfman disease. H&E stain.
LM	sinus histiocytosis with histiocytes have a singular large round nucleus (~2x the size of a lymphocyte) with a prominent nucleolus (visible with 10x objective); emperipolesis
LM DDx	other specific histiocytoses (Langerhans cell histiocytosis, Erdheim-Chester disease), specific infections (rhinoscleroma, xanthogranulomatous pyelonephritis), sinus histiocytosis
IHC	CD68 -ve, S-100 +ve, CD1a -ve
Site	lymph nodes - see lymph node pathology
Prevalence	rare

It is also known as sinus histiocytosis with massive lymphadenopathy, abbreviated SHML.

General

• Super rare.
• Prognosis - good.

Clinical findings:

• Fever.
• Leukocytosis with neutrophilia.
• Polyclonal gammaglobulinemia.

Microscopic

Features:

• Sinus histiocytosis:
  • Histiocytes - abundant.
    • Singular large round nuclei ~2x the size of resting lymphocyte.
      • Prominent nucleolus - visible with 10x objective.
    • Abundant cytoplasm.
• Emperipolesis (from Greek: em = inside, peri = around, polemai = wander about):
  • Histiocytes contain other whole cells: neutrophils, lymphocytes, plasma cells.
    • The "eaten" cell is within a vacuole; thus, it should have a clear halo around it.
    • Thought to be related to peripolesis; the attachment of a cell to another.

DDx:

• Other specific histiocytoses:
  • Langerhans cell histiocytosis.
  • Erdheim-Chester disease.
• Infection, e.g. rhinoscleroma (nasopharynx), xanthogranulomatous pyelonephritis.
• Sinus histiocytosis.
• Xanthomatous change.

Images

• 

  Emperipolesis in SHML (WC)

• 

  Rosai-Dorfman disease (WC)

• 

  Rosai-Dorfman disease - S-100 showing emperipolesis (WC)

www:

• RDD - case 1 - several images (upmc.edu).
• RDD - case 2 - several images of breast (upmc.edu).
• RDD - case 3 - several images (upmc.edu).
• RDD - case 4 - several images (upmc.edu).

IHC

• CD68 +ve.
• S-100 +ve.
  • Useful for seeing emperipolesis.
• CD1a -ve.
  • CD1a +ve in Langerhans cell histiocytosis.

See also

• Lymph node pathology.
• Sinus histiocytosis.
• Dermatopathic lymphadenopathy.
• Histiocytoses.