Castleman disease
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Castleman disease, abbreviated CD, is a rare pathology of the lymph node.
| 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 |
It is also known as angiofollicular lymph node hyperplasia and giant lymph node hyperplasia.[1]
General
- Benign.
- Hyaline vascular variant (classic Castleman disease) - a pathology of the follicular dendritic cells.[2]
Classification
By site of involvement:
- Unicentric (one lymph node or a cluster of nodes at one site)
- ~75% hyaline vascular variant
- ~25% Plasma cell variant
- Multicentric often associated with splenomegaly (75%) and hepatomegaly (50%)
- >90% plasma cell variant
- <10% hyaline vascular variant
CD is grouped by histologic appearance:[3]
- Hyaline vascular (HV) variant (described by Castleman).
- Usually unicentric.
- Typically mediastinal or axial.
- More common than plasma cell variant; represents 80-90% of CD cases.
- May be associated with follicular dendritic cell neoplasia.[4]
- Plasma cell (PC) variant.
- Usually multicentric, may be unicentric.
- Abundant plasma cells.
- 50-60% associated with HHV-8 infection (the same virus implicated in Kaposi's sarcoma). The virus produces an IL-6 analogue which drives many of the systemic features.
Some divide the multicentric pattern into
- POEMS-associated CD
- HHV8+ CD
- HIV+
- HIV-
- HHV8- CD, further sub-divided into
- TAFRO syndrome associated
- Others
Notes:
- The subclassification of CD is in some flux. Some authors advocate splitting-out HHV-8 and multicentric as separate subtypes.[5]
Microscopic
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, so called "twinning".
- Hyaline material (pink acellular stuff on H&E) in germinal center.
- Sinuses effaced (lost).
- Mitoses absent.
Images
CD HVV - "lollipop" sign - high mag. (WC)
CD HVV - showing expanded mantle zone - intermed. mag. (WC)
www:
Plasma cell variant
Features:[7]
- Interfollicular sheets of plasma cells - key feature.
- Active germinal centers - mitoses present.
- Sinus preserved.
- In HHV8-negative cases, several other conditions can give a Castleman-like appearance, listed comprehensively in the consensus criteria for HHV8-negative CD:
- Autoimmune diseases
- Infections
- Malignancies
IHC
Hyaline-vascular variant:
- Stains to exclude mantle cell lymphoma:
- Cyclin D1.
Plasma cell variant:
- HHV-8 +ve.
See also
References
- ↑ URL: http://www.mayoclinic.com/health/castleman-disease/DS01000. Accessed on: 17 June 2010.
- ↑ 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.
- ↑ Ioachim, Harry L; Medeiros, L. Jeffrey (2008). Ioachim's Lymph Node Pathology (4th ed.). Lippincott Williams & Wilkins. pp. 228. ISBN 978-0781775960.
- ↑ 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.
- ↑ 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.
- ↑ URL: http://www.ispub.com/journal/the_internet_journal_of_otorhinolaryngology/volume_9_number_2_11/article/a_rare_case_of_castleman_s_disease_presenting_as_cervical_neck_mass.html. Accessed on: 15 June 2010.
- ↑ 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.
- ↑ "Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease". Blood 129 (12): 1658–1668. March 2017. doi:10.1182/blood-2016-11-748855. PMC 5364343. PMID 28100459. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5364343/.
- ↑ "International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease". Blood 129 (12): 1646–1657. March 2017. doi:10.1182/blood-2016-10-746933. PMC 5364342. PMID 28087540. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5364342/.