Difference between revisions of "Hodgkin lymphoma"

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Separate page for NLPHL
(Separate page for NLPHL)
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| ClinDDx    =
| ClinDDx    =
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{{ Infobox diagnosis
'''Hodgkin [[lymphoma]]''', abbreviated '''HL''', is a haematological [[malignancy]]. If not otherwise specified, Hodgkin lymphoma generally refers to classical Hodgkin lymphoma (cHL) rather than less common [[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.
| Name      = Nodular lymphocyte-predominant Hodgkin lymphoma
 
| Image      = Popcorn_cell_in_nodular_lymphocyte_predominant_Hodgkin_lymphoma_-_very_high_mag_cropped.jpg
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.
| Width      =
| Caption    = Popcorn cell in nodular lymphocyte-predominant Hodgkin lymphoma
| Micro      = Popcorn cells (relatively) small (compared to classic RSCs) - have lobulated nucleus (key feature), small nucleoli; subtle nodularity at low power
| Subtypes  = none
| LMDDx      = [[diffuse large B cell lymphoma]] (esp. ''T-cell/histiocytic-rich LBCL''), [[anaplastic large cell lymphoma]],
B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Hodgkin lymphoma, classical Hodgkin lymphoma
| Stains    =
| IHC        = LCA (CD45) +ve, CD20 +ve, CD10 +ve, Bcl-6 +ve, EMA +ve, CD30 -ve, CD15 -ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Site      = usu. [[lymph node]]
| Assdx      =
| Syndromes  =
| Clinicalhx =
| Signs      = lymphadenopathy
| Symptoms  =
| Prevalence = uncommon
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  = good
| Other      =
| ClinDDx    =
}}
'''Hodgkin [[lymphoma]]''', abbreviated '''HL''', is a [[malignancy]] that often afflicts people in the prime of their life. 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 (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.
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.
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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.


==Hodgkin lymphoma subtypes==
===Classic HL sub-types===
Types:<ref name=Ref_WMSP567/>
====Subtypes of classic HL====
*Classical HL (CHL) - ~95% of HL.
There are four CHL subtypes:<ref name=Ref_WMSP567/>
*Nodular lymphocyte-predominant HL (NLPHL) - ~5% of HL.
#Nodular sclerosis CHL - ~70% of CHL.
#*Mixed cellular background - T cell, plasma cells, eosinophils, neutrophils and histiocytes.
#*Nodular sclerosing fibrosis - thick strands fibrosis.
#Mixed cellularity CHL - ~20-25% of CHL.
#*Like nodular sclerosis - but no fibrosis.
#*May be associated with [[HIV]] infection.<ref name=pmid20138008>{{cite journal |author=Sissolak G, Sissolak D, Jacobs P |title=Human immunodeficiency and Hodgkin lymphoma |journal=Transfus. Apher. Sci. |volume=42 |issue=2 |pages=131–9 |year=2010 |month=April |pmid=20138008 |doi=10.1016/j.transci.2010.01.008 |url=}}</ref>
#Lymphocyte-rich CHL - rare.
#*T lymphocytes only (no mix of cells).
#Lymphocyte-depleted CHL - rare.
#*May be associated with HIV infection.<ref name=pmid20138008/>


===Classic HL===
Memory device:
*Four types of classic HL (see below).
*The subtypes prevalence is in reverse alphabetical order.
 
===NLPHL===
*AKA ''lympho-histiocytic variant''.
*Abbreviated ''NLPHL''.
*Different IHC and morphologic appearance than classic HL.
*Significant risk for transformation into [[diffuse large B cell lymphoma]] (DLBCL); 10-year cumulative transformation rate (to DLBCL) in one study was 12%.<ref name=pmid20029973 >{{cite journal |author=Biasoli I, Stamatoullas A, Meignin V, ''et al.'' |title=Nodular, lymphocyte-predominant Hodgkin lymphoma: a long-term study and analysis of transformation to diffuse large B-cell lymphoma in a cohort of 164 patients from the Adult Lymphoma Study Group |journal=Cancer |volume=116 |issue=3 |pages=631–9 |year=2010 |month=February |pmid=20029973 |doi=10.1002/cncr.24819 |url=}}</ref>


==Gross==
==Gross==
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Image:Hodgkin_lymphoma_%281%29_mixed_cellulary_type.jpg | HL mixed cellularity. (WC)
Image:Hodgkin_lymphoma_%281%29_mixed_cellulary_type.jpg | HL mixed cellularity. (WC)
</gallery>
</gallery>
====Subtypes of classic HL====
There are four CHL subtypes:<ref name=Ref_WMSP567/>
#Nodular sclerosis CHL - ~70% of CHL.
#*Mixed cellular background - T cell, plasma cells, eosinophils, neutrophils and histiocytes.
#*Nodular sclerosing fibrosis - thick strands fibrosis.
#Mixed cellularity CHL - ~20-25% of CHL.
#*Like nodular sclerosis - but no fibrosis.
#*May be associated with [[HIV]] infection.<ref name=pmid20138008>{{cite journal |author=Sissolak G, Sissolak D, Jacobs P |title=Human immunodeficiency and Hodgkin lymphoma |journal=Transfus. Apher. Sci. |volume=42 |issue=2 |pages=131–9 |year=2010 |month=April |pmid=20138008 |doi=10.1016/j.transci.2010.01.008 |url=}}</ref>
#Lymphocyte-rich CHL - rare.
#*T lymphocytes only (no mix of cells).
#Lymphocyte-depleted CHL - rare.
#*May be associated with HIV infection.<ref name=pmid20138008/>
Memory device:
*The subtypes prevalence is in reverse alphabetical order.
===Nodular lymphocyte predominant Hodgkin lymphoma===
Features (nodular lymphocyte-predominant Hodgkin's lymphoma):
*''Popcorn cell'' (previously known as ''Lymphocytic & histiocytic'' cell (L&H cell)<ref name=pmid9499174>{{cite journal |author=Küppers R, Rajewsky K, Braeuninger A, Hansmann ML |title=L&H cells in lymphocyte-predominant Hodgkin's disease |journal=N. Engl. J. Med. |volume=338 |issue=11 |pages=763–4; author reply 764–5 |year=1998 |month=March |pmid=9499174 |doi=10.1056/NEJM199803123381113 |url=}}</ref>) - variant of RSC:
**Cells (relatively) small (compared to classic RSCs).
**Lobulated nucleus - '''key feature'''.
**Small nucleoli.
*Subtle nodularity at low power (2.5x or 5x objective).


====Images (NLPHL)====
www:
*[http://webpathology.com/image.asp?case=388&n=16 Popcorn cell (webpathology.com)].
<gallery>
Image:Popcorn_cell_in_nodular_lymphocyte_predominant_Hodgkin_lymphoma_-_very_high_mag_cropped.jpg | Popcorn cell. (WC)
</gallery>
===DDx both CHL & NLPHL===
===DDx both CHL & NLPHL===
*CHL/NLPHL.
*CHL/NLPHL.
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