Difference between revisions of "Castleman disease"
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Castleman_disease_-_high_mag.jpg | |||
| Width = | |||
| Caption = Castleman disease (hyaline-vascular variant). [[H&E stain]]. | |||
| Synonyms = | |||
| Micro = | |||
| Subtypes = hyaline-vascular variant (HVV), plasma cell variant (PCV) | |||
| LMDDx = HVV: [[mantle cell lymphoma]] | |||
| Stains = | |||
| IHC = HVV: cyclin D1 -ve, other stains to exclude lymphoma; PCV: HHV-8 +ve | |||
| EM = | |||
| Molecular = | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[lymph node]] - see ''[[lymph node pathology]]'' | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = rare | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = | |||
| Other = | |||
| ClinDDx = | |||
| Tx = | |||
}} | |||
'''Castleman disease''', abbreviated '''CD''', is a rare [[Lymph node pathology|pathology of the lymph node]]. | '''Castleman disease''', abbreviated '''CD''', is a rare [[Lymph node pathology|pathology of the lymph node]]. | ||
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==General== | ==General== | ||
*Benign. | *Benign. | ||
*Hyaline vascular variant - a pathology of the follicular dendritic cells.<ref>{{Cite journal | last1 = Cokelaere | first1 = K. | last2 = Debiec-Rychter | first2 = M. | last3 = De Wolf-Peeters | first3 = C. | last4 = Hagemeijer | first4 = A. | last5 = Sciot | first5 = R. | title = Hyaline vascular Castleman's disease with HMGIC rearrangement in follicular dendritic cells: molecular evidence of mesenchymal tumorigenesis. | journal = Am J Surg Pathol | volume = 26 | issue = 5 | pages = 662-9 | month = May | year = 2002 | doi = | PMID = 11979097 }}</ref> | *Hyaline vascular variant (classic Castleman disease) - a pathology of the follicular dendritic cells.<ref>{{Cite journal | last1 = Cokelaere | first1 = K. | last2 = Debiec-Rychter | first2 = M. | last3 = De Wolf-Peeters | first3 = C. | last4 = Hagemeijer | first4 = A. | last5 = Sciot | first5 = R. | title = Hyaline vascular Castleman's disease with HMGIC rearrangement in follicular dendritic cells: molecular evidence of mesenchymal tumorigenesis. | journal = Am J Surg Pathol | volume = 26 | issue = 5 | pages = 662-9 | month = May | year = 2002 | doi = | PMID = 11979097 }}</ref> | ||
===Classification=== | ===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:<ref name=Ref_ILNP228>{{Ref ILNP|228}}</ref> | CD is grouped by histologic appearance:<ref name=Ref_ILNP228>{{Ref ILNP|228}}</ref> | ||
#Hyaline vascular (HV) variant (described by Castleman). | #Hyaline vascular (HV) variant (described by Castleman). | ||
| Line 17: | Line 56: | ||
#*Usually multicentric, may be unicentric. | #*Usually multicentric, may be unicentric. | ||
#*Abundant plasma cells. | #*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: | Notes: | ||
| Line 24: | Line 72: | ||
==Microscopic== | ==Microscopic== | ||
===Hyaline-vascular variant=== | ===Hyaline-vascular variant=== | ||
Features:<ref>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 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.</ref><ref name=Ref_ILNP236>{{Ref ILNP|236}}</ref> | Features:<ref>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 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.</ref><ref name=Ref_ILNP236>{{Ref ILNP|236}}</ref><ref name=pmid28100459>{{cite journal |authors=Yu L, Tu M, Cortes J, Xu-Monette ZY, Miranda RN, Zhang J, Orlowski RZ, Neelapu S, Boddu PC, Akosile MA, Uldrick TS, Yarchoan R, Medeiros LJ, Li Y, Fajgenbaum DC, Young KH |title=Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease |journal=Blood |volume=129 |issue=12 |pages=1658–1668 |date=March 2017 |pmid=28100459 |pmc=5364343 |doi=10.1182/blood-2016-11-748855 |url=}}</ref> | ||
*Pale concentric (expanded) mantle zone lymphocytes - '''key feature'''. | *Pale concentric (expanded) mantle zone lymphocytes - '''key feature'''. | ||
**"Regressed follicles" - germinal center (pale area) is small. | **"Regressed follicles" - germinal center (pale area) is small. | ||
*"Lollipops": | *"Lollipops": | ||
**Germinal centers fed by prominent (radially penetrating sclerotic) vessels; lollipop-like appearance. | **Germinal centers fed by prominent (radially penetrating sclerotic) vessels; lollipop-like appearance. | ||
*Two germinal centers in one follicle. | *Two germinal centers in one follicle, so called "twinning". | ||
*Hyaline material (pink acellular stuff on H&E) in germinal center. | *Hyaline material (pink acellular stuff on H&E) in germinal center. | ||
*Sinuses effaced (lost). | *Sinuses effaced (lost). | ||
| Line 47: | Line 96: | ||
*Interfollicular sheets of plasma cells - '''key feature'''. | *Interfollicular sheets of plasma cells - '''key feature'''. | ||
*Active germinal centers - mitoses present. | *Active germinal centers - mitoses present. | ||
*Sinus | *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: | |||
<ref name=pmid28087540>{{cite journal |authors=Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, Simpson D, Liu AY, Menke D, Chandrakasan S, Lechowicz MJ, Wong RS, Pierson S, Paessler M, Rossi JF, Ide M, Ruth J, Croglio M, Suarez A, Krymskaya V, Chadburn A, Colleoni G, Nasta S, Jayanthan R, Nabel CS, Casper C, Dispenzieri A, Fosså A, Kelleher D, Kurzrock R, Voorhees P, Dogan A, Yoshizaki K, van Rhee F, Oksenhendler E, Jaffe ES, Elenitoba-Johnson KS, Lim MS |title=International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease |journal=Blood |volume=129 |issue=12 |pages=1646–1657 |date=March 2017 |pmid=28087540 |pmc=5364342 |doi=10.1182/blood-2016-10-746933 |url=}}</ref> | |||
**Autoimmune diseases | |||
**Infections | |||
**Malignancies | |||
==IHC== | ==IHC== | ||
Latest revision as of 17:22, 25 March 2021
| 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]
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.
- ↑ Yu L, Tu M, Cortes J, Xu-Monette ZY, Miranda RN, Zhang J, Orlowski RZ, Neelapu S, Boddu PC, Akosile MA, Uldrick TS, Yarchoan R, Medeiros LJ, Li Y, Fajgenbaum DC, Young KH (March 2017). "Clinical and pathological characteristics of HIV- and HHV-8-negative Castleman disease". Blood 129 (12): 1658–1668. doi:10.1182/blood-2016-11-748855. PMC 5364343. PMID 28100459. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5364343/.
- ↑ Fajgenbaum DC, Uldrick TS, Bagg A, Frank D, Wu D, Srkalovic G, Simpson D, Liu AY, Menke D, Chandrakasan S, Lechowicz MJ, Wong RS, Pierson S, Paessler M, Rossi JF, Ide M, Ruth J, Croglio M, Suarez A, Krymskaya V, Chadburn A, Colleoni G, Nasta S, Jayanthan R, Nabel CS, Casper C, Dispenzieri A, Fosså A, Kelleher D, Kurzrock R, Voorhees P, Dogan A, Yoshizaki K, van Rhee F, Oksenhendler E, Jaffe ES, Elenitoba-Johnson KS, Lim MS (March 2017). "International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease". Blood 129 (12): 1646–1657. doi:10.1182/blood-2016-10-746933. PMC 5364342. PMID 28087540. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5364342/.