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Difference between revisions of "Hodgkin lymphoma"
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| Assdx = | | Assdx = | ||
| Syndromes = | | Syndromes = | ||
| Clinicalhx = | | Clinicalhx = young adults, older adults (bimodal distribution) | ||
| Signs = | | Signs = lymphadenopathy - usu. neck, +/-[[B symptoms]] (fever, night sweats, weight loss) | ||
| Symptoms = | | Symptoms = | ||
| Prevalence = common | | Prevalence = common | ||
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| Rads = | | Rads = | ||
| Endoscopy = | | Endoscopy = | ||
| Prognosis = | | Prognosis = usu. good, dependent on stage | ||
| Other = | | Other = | ||
| ClinDDx = | | ClinDDx = | ||
}} | }} | ||
'''Hodgkin [[lymphoma]]''', abbreviated '''HL''', is a haematological [[malignancy]]. If not otherwise specified, Hodgkin lymphoma generally refers to classical Hodgkin lymphoma (CHL) rather than [[nodular lymphocyte-predominant Hodgkin lymphoma]] (NLPHL). The latter accounts for only about 5% of the loose label of Hodgkin lymphoma<ref name=Ref_WMSP567/> and shows a sufficiently different biology and immunophenotype that is essentially a different disease (see [[nodular lymphocyte-predominant Hodgkin lymphoma|main article on NLPHL]]). The common feature is large atypical cells: Hodgkin/Reed-Sternberg cells in the case of classical Hodgkin lymphoma and "popcorn"/lymphohistiocytic/L&H cells in NLPHL. However, at least in typical cases, there are morphological and immunophenotypic differences. | |||
Classical Hodgkin lymphoma has a bi-modal distribution, afflicting young adults and with a further peak in incidence in middle-age. Fortunately, it usually has a good prognosis. | |||
Pathologists say "... it is both the easiest and hardest diagnosis to make." The reason for this is: the diagnosis depends on finding Reed-Sternberg cells; if they are obvious the diagnosis is easy... if you can't find 'em and an alternative diagnosis is not apparent -- you wonder whether you're missing them. | Pathologists say "... it is both the easiest and hardest diagnosis to make." The reason for this is: the diagnosis depends on finding Reed-Sternberg cells (or Popcorn cells); if they are obvious the diagnosis is easy... if you can't find 'em and an alternative diagnosis is not apparent -- you wonder whether you're missing them. | ||
==General== | ==General== | ||
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*Usually good. | *Usually good. | ||
*Unlike non-Hodgkin lymphomas, it spreads in a predictable pattern; thus, staging plays an important role in determining the therapy.<ref name=PCPBoD8_315>{{Ref PCPBoD8|315}}</ref> | *Unlike non-Hodgkin lymphomas, it spreads in a predictable pattern; thus, staging plays an important role in determining the therapy.<ref name=PCPBoD8_315>{{Ref PCPBoD8|315}}</ref> | ||
*Clinically classified into early favourable, early unfavourable and late/advanced disease. | |||
== | ===Classic HL sub-types=== | ||
====Subtypes of classic HL==== | ====Subtypes of classic HL==== | ||
There are four CHL subtypes:<ref name=Ref_WMSP567/> | There are four CHL subtypes:<ref name=Ref_WMSP567/> | ||
| Line 133: | Line 66: | ||
*The subtypes prevalence is in reverse alphabetical order. | *The subtypes prevalence is in reverse alphabetical order. | ||
== | ==Gross== | ||
Location: | |||
*'' | *Almost always arises from a [[lymph node]] - classically in the neck, but may be in the axilla and mediastinum | ||
* | *Spleen may be involved | ||
** | *Bone marrow involvement is unusual (~5% of cases, higher in HIV-associated cases), so bone marrow assessment is usually not performed | ||
** | *''Extranodal Hodgkin lymphoma'' is (case report) rare.<ref name=pmid11100066>{{Cite journal | last1 = Vadmal | first1 = MS. | last2 = LaValle | first2 = GP. | last3 = DeYoung | first3 = BR. | last4 = Frankel | first4 = WL. | last5 = Marsh | first5 = WL. | title = Primary localized extranodal hodgkin disease of the transverse colon. | journal = Arch Pathol Lab Med | volume = 124 | issue = 12 | pages = 1824-7 | month = Dec | year = 2000 | doi = 10.1043/0003-9985(2000)1241824:PLEHDO2.0.CO;2 | PMID = 11100066 }}</ref> | ||
* | |||
==Microscopic== | |||
Defined by ''Reed-Sternberg cells'' (RSCs). Morphologically similar mononuclear cells are known as Hodgkin cells. RSCs are: | |||
*Large binucleated cell (>= 45 micrometres).<ref name=Ref_PCPBoD8_329>{{Ref PCPBoD8|329}}</ref> | |||
**May be multinucleated. | |||
**May have a horseshoe-like shape. | |||
*[[Macronucleolus]] - approximately the size of a RBC (~8 micrometers). | |||
*Well-defined cell border. | |||
*Abundant cytoplasm. | |||
RSC may show peri-cellular clearing, making the cells appear within a space. These are called lacunar cells (as they are in a "lake"). Apoptotic RSC may show pyknotic nuclei and scant eosinophilic cytoplasm and are sometimes known as "mummified" cells. | |||
===Images (classic HL)=== | |||
<gallery> | <gallery> | ||
Image: | Image:CHL mummified cell x40.jpg | "Mummified" RSC. (WC) | ||
Image:CHL lacunar cell x40.jpg | "Lacunar cell". (WC) | |||
Image:16S14098 cHL multinucleate HRS cell x40c.jpg | Multinucleate RSC. (WC) | |||
Image:Hodgkin_lymphoma_cytology_large.jpg | HL mixed cellularity - cytology. (WC) | |||
Image:Hodgkin_lymphoma_cytology_small.jpg | HL mixed cellularity - cytology. (WC) | |||
Image:Hodgkin_lymphoma_%281%29_mixed_cellulary_type.jpg | HL mixed cellularity. (WC) | |||
</gallery> | </gallery> | ||
===DDx both CHL & NLPHL=== | ===DDx both CHL & NLPHL=== | ||
*CHL/NLPHL. | *CHL/NLPHL. | ||
*[[Diffuse large B cell lymphoma]] (DLBCL), esp. ''T-cell/histiocytic-rich LBCL''. | *[[Diffuse large B cell lymphoma]] (DLBCL), esp. ''T-cell/histiocytic-rich LBCL''. | ||
*[[Anaplastic large cell lymphoma]] (ALCL). | *[[Anaplastic large cell lymphoma]] (ALCL). | ||
*B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma.<ref name=pmid22222636>{{Cite journal | last1 = Gualco | first1 = G. | last2 = Natkunam | first2 = Y. | last3 = Bacchi | first3 = CE. | title = The spectrum of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma: a description of 10 cases. | journal = Mod Pathol | volume = | issue = | pages = | month = Jan | year = 2012 | doi = 10.1038/modpathol.2011.200 | PMID = 22222636 | URL = http://www.nature.com/modpathol/journal/vaop/ncurrent/full/modpathol2011200a.html }}</ref> | *B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma.<ref name=pmid22222636>{{Cite journal | last1 = Gualco | first1 = G. | last2 = Natkunam | first2 = Y. | last3 = Bacchi | first3 = CE. | title = The spectrum of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma: a description of 10 cases. | journal = Mod Pathol | volume = | issue = | pages = | month = Jan | year = 2012 | doi = 10.1038/modpathol.2011.200 | PMID = 22222636 | URL = http://www.nature.com/modpathol/journal/vaop/ncurrent/full/modpathol2011200a.html }}</ref> (typically in cases of numerous large atypical cells where the morphology and immunophenotype do not neatly fit into either DLBCL or CHL). | ||
==IHC== | ==IHC== | ||
Abbreviated panel:<ref name=Ref_WMSP568>{{Ref_WMSP|568}}</ref> | Abbreviated panel:<ref name=Ref_WMSP568>{{Ref_WMSP|568}}</ref> | ||
*CD30 Reed-Sternberg cells (RSCs) +ve ~98% | *CD30 Reed-Sternberg cells (RSCs) +ve ~98% (beware of mis-interpreting CD30+ activated lymphoid cells) | ||
*CD15 Reed-Sternberg cells +ve ~80%, stains neutrophils. | *CD15 Reed-Sternberg cells +ve ~80%, stains neutrophils. | ||
*Both CD30 and CD15 are classically positive in a membranous and Golgi pattern | |||
*MUM1 +ve | |||
*CD45 '''often negative''' in RSCs. | *CD45 '''often negative''' in RSCs. | ||
*CD20 may stain RSCs. | *CD20 may stain RSCs (usually negative, but can be weak). | ||
*PAX5 +ve | *PAX5 +ve, though said to be weaker than background normal B-cells<ref name=Ref_APBR683>{{Ref APBR|683}}</ref> | ||
Additional - for completeness: | Additional - for completeness: | ||
*CD3 (T lymphocytes) | *CD3 (T lymphocytes) - negative in RSCs | ||
*OCT2/BOB1 negative (co-transcription factors for immunoglobulin production, one or the other is usually negative) | |||
*40% are EBV positive. | |||
NLPHL IHC '''differs''' from the classical HL:<ref name=Ref_APBR683>{{Ref APBR|683}}</ref> | NLPHL IHC '''differs''' from the classical HL:<ref name=Ref_APBR683>{{Ref APBR|683}}</ref> | ||
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*CD10 +ve. | *CD10 +ve. | ||
*Bcl-6 +ve. | *Bcl-6 +ve. | ||
*EMA +ve | *[[EMA]] +ve (40-50%) | ||
*CD30 -ve | *CD30 -ve | ||
*CD15 -ve. | *CD15 -ve. | ||
=== | ===A panel=== | ||
{| class="wikitable" | {| class="wikitable" | ||
|Antibody || NLPHL || CHL | |Antibody || NLPHL || CHL | ||
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|CD20 || +ve || -ve | |CD20 || +ve || -ve | ||
|- | |- | ||
|BCL6 || || | |BCL6 || +ve || -ve | ||
|- | |- | ||
|MUM1<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/601900 http://www.ncbi.nlm.nih.gov/omim/601900]. Accessed on: 10 August 2010.</ref> || -ve || | |MUM1<ref>URL: [http://www.ncbi.nlm.nih.gov/omim/601900 http://www.ncbi.nlm.nih.gov/omim/601900]. Accessed on: 10 August 2010.</ref> || -ve || +ve | ||
|- | |- | ||
|CD30 || -ve || +ve (most sensitive). | |CD30 || -ve || +ve (most sensitive). | ||
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|CD21 || networks present || no networks | |CD21 || networks present || no networks | ||
|- | |- | ||
|CD23 | |[[CD23]] || networks present || no networks | ||
|- | |- | ||
|OCT-2 || +ve || -ve | |OCT-2 || +ve || -ve | ||
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|4 unstained || || | |4 unstained || || | ||
|} | |} | ||
==Sign out== | |||
===Suggestive FNA=== | |||
<pre> | |||
Lymph Node, Right Neck, FNA: | |||
- Large binucleated and multinucleated cells with macronucleoli in | |||
a background of abundant lymphocytes, histiocytes, rare eosinophils. | |||
Comment: | |||
A cell block is not available for further work-up. The findings raise the possibility of Hodgkin's lymphoma. | |||
A further biopsy is required for the diagnosis. | |||
</pre> | |||
==See also== | ==See also== | ||