Difference between revisions of "Castleman disease"

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Castleman disease (view source)

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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).
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#*Usually multicentric, may be unicentric.
#*Usually multicentric, may be unicentric.
#*Abundant plasma cells.
#*Abundant plasma cells.
#*Associated with [[HHV-8]] infection (the same virus implicated in ''Kaposi's sarcoma'').
#*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:
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==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).
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*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 perserved.
*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==
Michael
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