Castleman disease

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Castleman disease
Diagnosis in short

Castleman disease (hyaline-vascular variant). H&E stain.
Subtypes hyaline-vascular variant (HVV), plasma cell variant (PCV)
LM DDx HVV: mantle cell lymphoma
IHC HVV: cyclin D1 -ve, other stains to exclude lymphoma; PCV: HHV-8 +ve
Site lymph node - see lymph node pathology

Prevalence rare

Castleman disease, abbreviated CD, is a rare pathology of the lymph node.

It is also known as angiofollicular lymph node hyperplasia and giant lymph node hyperplasia.[1]


  • Benign.
  • Hyaline vascular variant (classic Castleman disease) - a pathology of the follicular dendritic cells.[2]


CD is grouped by histologic appearance:[3]

  1. Hyaline vascular (HV) variant (described by Castleman).
  2. Plasma cell (PC) variant.
    • Usually multicentric, may be unicentric.
    • Abundant plasma cells.
    • Associated with HHV-8 infection (the same virus implicated in Kaposi's sarcoma).


  • The subclassification of CD is in some flux. Some authors advocate splitting-out HHV-8 and multicentric as separate subtypes.[5]


Hyaline-vascular variant


  • Pale concentric (expanded) mantle zone lymphocytes - key feature.
    • "Regressed follicles" - germinal center (pale area) is small.
  • "Lollipops":
    • Germinal centers fed by prominent (radially penetrating sclerotic) vessels; lollipop-like appearance.
  • Two germinal centers in one follicle.
  • Hyaline material (pink acellular stuff on H&E) in germinal center.
  • Sinuses effaced (lost).
  • Mitoses absent.



Plasma cell variant


  • Interfollicular sheets of plasma cells - key feature.
  • Active germinal centers - mitoses present.
  • Sinus perserved.


Hyaline-vascular variant:

Plasma cell variant:

  • HHV-8 +ve.

See also


  1. URL: Accessed on: 17 June 2010.
  2. Cokelaere, K.; Debiec-Rychter, M.; De Wolf-Peeters, C.; Hagemeijer, A.; Sciot, R. (May 2002). "Hyaline vascular Castleman's disease with HMGIC rearrangement in follicular dendritic cells: molecular evidence of mesenchymal tumorigenesis.". Am J Surg Pathol 26 (5): 662-9. PMID 11979097.
  3. Ioachim, Harry L; Medeiros, L. Jeffrey (2008). Ioachim's Lymph Node Pathology (4th ed.). Lippincott Williams & Wilkins. pp. 228. ISBN 978-0781775960.
  4. Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 596. ISBN 978-0781765275.
  5. Cronin, DM.; Warnke, RA. (Jul 2009). "Castleman disease: an update on classification and the spectrum of associated lesions.". Adv Anat Pathol 16 (4): 236-46. doi:10.1097/PAP.0b013e3181a9d4d3. PMID 19546611.
  6. URL: Accessed on: 15 June 2010.
  7. 7.0 7.1 Ioachim, Harry L; Medeiros, L. Jeffrey (2008). Ioachim's Lymph Node Pathology (4th ed.). Lippincott Williams & Wilkins. pp. 236. ISBN 978-0781775960.