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Anaplastic large cell lymphoma (view source)
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Anaplastic_large_cell_lymphoma_-_cropped_-_very_high_mag.jpg | |||
| Width = | |||
| Caption = Anaplastic large cell lymphoma. [[H&E stain]]. | |||
| Synonyms = | |||
| Micro = large cells with eosinophilic cytoplasm, Hallmark cells ("horseshoe-shaped or donut-shaped nucleus + eosinophilic paranuclear region") | |||
| Subtypes = | |||
| LMDDx = [[Hodgkin's lymphoma]], poorly differentiated [[carcinoma]] | |||
| Stains = | |||
| IHC = CD30 +ve, ALK-1 -ve/+ve, CD45 +ve, CD4 +ve, CD3 -ve/+ve, CD7 -ve/+ve, EMA +ve | |||
| EM = | |||
| Molecular = t(2,5)(p23;q35) | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[skin]], [[lymph node]], other | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = uncommon | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = good to poor, dependent on ALK status and site | |||
| Other = | |||
| ClinDDx = | |||
| Tx = | |||
}} | |||
'''Anaplastic large cell lymphoma''', abbreviated '''ALCL''', is an uncommon large cell [[lymphoma]]. | |||
==General== | |||
*May look a lot like a carcinoma. | |||
**Often subcapsular in LNs. | |||
*Usually T-cell derived. | |||
*May be isolated to the [[skin]] - good prognosis. | |||
Subtypes: | |||
* Systemic ALCL. | |||
* Cutaneous ALCL -- ALK -ve. | |||
ALK IHC - systemic form: | |||
* +ve = good prognosis (generally a disease of children, teenagers and young adults) | |||
* -ve = bad prognosis | |||
==Microscopic== | |||
Features: | |||
*Large cells with eosinophilic cytoplasm. | |||
*Usually appear cohesive. | |||
*May be subcapsular in a [[lymph node]] and mimic a carcinoma. | |||
*''Hallmark cells'' = "horseshoe-shaped or [[donut cell|donut-shaped nucleus]] + eosinophilic paranuclear region"<ref name=pmid17941004>{{Cite journal | last1 = Rapkiewicz | first1 = A. | last2 = Wen | first2 = H. | last3 = Sen | first3 = F. | last4 = Das | first4 = K. | title = Cytomorphologic examination of anaplastic large cell lymphoma by fine-needle aspiration cytology. | journal = Cancer | volume = 111 | issue = 6 | pages = 499-507 | month = Dec | year = 2007 | doi = 10.1002/cncr.23120 | PMID = 17941004 | url=http://onlinelibrary.wiley.com/doi/10.1002/cncr.23120/full}}</ref><ref name=pmid12419758>{{Cite journal | last1 = Ponzoni | first1 = M. | last2 = Terreni | first2 = MR. | last3 = Ciceri | first3 = F. | last4 = Ferreri | first4 = AJ. | last5 = Gerevini | first5 = S. | last6 = Anzalone | first6 = N. | last7 = Valle | first7 = M. | last8 = Pizzolito | first8 = S. | last9 = Arrigoni | first9 = G. | title = Primary brain CD30+ ALK1+ anaplastic large cell lymphoma ('ALKoma'): the first case with a combination of 'not common' variants. | journal = Ann Oncol | volume = 13 | issue = 11 | pages = 1827-32 | month = Nov | year = 2002 | doi = | PMID = 12419758 }}</ref> - '''key feature'''. | |||
**The donut-shaped version is also known as a "wreath cell"<ref>{{Cite journal | last1 = Amin | first1 = HM. | last2 = Lai | first2 = R. | title = Pathobiology of ALK+ anaplastic large-cell lymphoma. | journal = Blood | volume = 110 | issue = 7 | pages = 2259-67 | month = Oct | year = 2007 | doi = 10.1182/blood-2007-04-060715 | PMID = 17519389 | url=http://bloodjournal.hematologylibrary.org/content/110/7/2259.full.html}}</ref> - large (multi-nucleated) cells with (morphologically) one toroidal-shaped nucleus. | |||
*ALK+ ALCL includes variants without the classical hallmark cells, e.g. small cell variant and lymphohistiocytic variant | |||
DDx: | |||
*[[Hodgkin's lymphoma]] | |||
*Anaplastic variants of other haematolymphoid malignancies, e.g. DLBCL or myeloma | |||
*Carcinoma | |||
*Melanoma | |||
===Images=== | |||
<gallery> | |||
Image: Anaplastic large cell lymphoma - high mag.jpg | ALCL - high mag. (WC) | |||
Image:Anaplastic_large_cell_lymphoma_-_very_high_mag.jpg | ALCL - very high mag. (WC) | |||
Image:Anaplastic_large_cell_lymphoma_-_cropped_-_very_high_mag.jpg | ALCL - cropped - very high mag. (WC) | |||
</gallery> | |||
www: | |||
*[http://path.upmc.edu/cases/case363.html ALCL - several images (upmc.edu)]. | |||
*[http://path.upmc.edu/cases/case557.html Small cell variant of ALCL - several images (upmc.edu)]. | |||
==IHC== | |||
Features: | |||
*CD30 +ve (usually strong and diffuse) | |||
*ALK-1 -ve/+ve; strongly supports ALCL Dx if +ve. | |||
*CD45 +ve. | |||
*CD4 +ve. | |||
*CD3 -ve/+ve. | |||
*CD7 -ve/+ve. | |||
*EMA +ve. | |||
*Cytotoxic markers (e.g. TIA, perforin) | |||
==Molecular== | |||
*In ALK+ ALCL, there is commonly an ALK1 rearrangement. | |||
**This may be ALK::NPM1, i.e. t(2,5)(p23;q35)<ref name=pmid8547653>{{cite journal |author=Lamant L, Meggetto F, al Saati T, ''et al.'' |title=High incidence of the t(2;5)(p23;q35) translocation in anaplastic large cell lymphoma and its lack of detection in Hodgkin's disease. Comparison of cytogenetic analysis, reverse transcriptase-polymerase chain reaction, and P-80 immunostaining |journal=Blood |volume=87 |issue=1 |pages=284–91 |year=1996 |month=January |pmid=8547653 |doi= |url=}}</ref> - combined nuclear and cytoplasmic ALK staining is a surrogate for this translocation. | |||
*Other non-NPM1 partners may occur. A ALK1 break-apart probe will detect these. | |||
==See also== | |||
*[[Lymphoma]]. | |||
*[[Diffuse large B-cell lymphoma]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Lymphoma]] | |||
[[Category:Diagnosis]] | |||