View source for Primary cutaneous CD30 positive anaplastic large cell lymphoma

{{Infobox medical condition
|name            = Primary cutaneous CD30 positive anaplastic large cell lymphoma
|synonyms        =  Primary cutaneous anaplastic large cell lymphoma.
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|specialty       = [[Dermatology]]
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'''Primary cutaneous anaplastic large cell lymphoma''' belongs to the group of cutaneous processes that are [[CD30]]+ [[Lymphoproliferative disorders|lymphoproliferative]] and are characterized by autoregressive, recurrent, single or multifocal ulcerating nodules. Single or localized nodules, [[Papule|papules]], or plaques are present in the majority of patients. However, a patient may have more than one lesion in up to 20% of cases.<ref name="case report">{{cite journal | last1=Oliveira | first1=Luciana Silveira Rabello de | last2=Nobrega | first2=Madeleyne Palhano | last3=Monteiro | first3=Maira Gomes | last4=Almeida | first4=Wagner Leite de | title=Primary cutaneous anaplastic large-cell lymphoma&nbsp;— case report | journal=Anais Brasileiros de Dermatologia | publisher=FapUNIFESP (SciELO) | volume=88 | issue=6 suppl 1 | year=2013 | issn=0365-0596 | doi=10.1590/abd1806-4841.20131731 | pages=132–135| pmid=24346900 | pmc=3875977 }}</ref>
For patients with localized lesions, the recommended courses of treatment include [[radiation therapy]], lesion removal, and/or low-dose [[methotrexate]].<ref name="Diamantidis Myrou 2011 pp. 1048–1055">{{cite journal | last1=Diamantidis | first1=Michael D. | last2=Myrou | first2=Athena D. | title=Perils and Pitfalls Regarding Differential Diagnosis and Treatment of Primary Cutaneous Anaplastic Large-Cell Lymphoma | journal=The Scientific World Journal | publisher=Hindawi Limited | volume=11 | year=2011 | issn=1537-744X | doi=10.1100/tsw.2011.80 | doi-access=free | pages=1048–1055| pmid=21552770 | pmc=5720019 }}</ref> Systemic polychemotherapy is indicated for the treatment of diseases that progress quickly or that are extracutaneous.<ref name="Diamantidis Papadopoulos Kaiafa Ntaios 2009 pp. 226–229">{{cite journal | last1=Diamantidis | first1=Michael D. | last2=Papadopoulos | first2=Athanasios | last3=Kaiafa | first3=Georgia | last4=Ntaios | first4=George | last5=Karayannopoulou | first5=Georgia | last6=Kostopoulos | first6=Ioannis | last7=Girtovitis | first7=Fotios | last8=Saouli | first8=Zoi | last9=Kontoninas | first9=Zisis | last10=Raptis | first10=Ioannis D. | last11=Savopoulos | first11=Christos | last12=Hatzitolios | first12=Apostolos | title=Differential diagnosis and treatment of primary, cutaneous, anaplastic large cell lymphoma: not always an easy task | journal=International Journal of Hematology | publisher=Springer Science and Business Media LLC | volume=90 | issue=2 | date=June 23, 2009 | issn=0925-5710 | doi=10.1007/s12185-009-0365-7 | pages=226–229| pmid=19548068 | s2cid=42622679 }}</ref>
== Signs and symptoms ==
Primary cutaneous anaplastic large cell lymphoma typically manifests as multifocal, solitary, or grouped nodules on the upper limbs that last for three to four weeks.<ref name="Brown Fernandez">{{cite journal | last1=Brown | first1=Ryanne A. | last2=Fernandez-Pol | first2=Sebastian | last3=Kim | first3=Jinah | title=Primary cutaneous anaplastic large cell lymphoma | journal=Journal of Cutaneous Pathology | volume=44 | issue=6 | date=2017 | issn=0303-6987 | doi=10.1111/cup.12937 | pages=570–577| pmid=28342276 }}</ref>
== Causes ==
The cause is complex and multifaceted. Only a small proportion of patients have [[CD30]]+ cells in normal [[lymphoid]] tissue. These are the cells that are thought to be the precursors of systemic [[Anaplastic large-cell lymphoma|anaplastic large cell lymphoma]]. It is believed that [[CD30]]'s interaction with its ligand, CD30L, mediates the disease's proliferative and anti-proliferative phases.<ref name="Brown Fernandez"/> The [[Chemokine receptor|chemokine receptors]] [[CCR8 (gene)|CCR8]] and [[CCR10]], which are implicated in skin localization, are also assumed to mediate cutaneous presentation through their expression. Another theory is that [[Langerhans cell]]-mediated antigen presentation chronically activates [[T cell]]s, leading to [[Cutaneous T-cell lymphoma|cutaneous T cell lymphomas]].<ref name="Moodley Nombona Mosam 2019 pp. 163–169">{{cite journal | last1=Moodley | first1=Nerissa | last2=Nombona | first2=Patiswa | last3=Mosam | first3=Anisa | title=Primary Cutaneous Anaplastic Large-Cell Lymphoma | journal=Dermatopathology | publisher=MDPI AG | volume=6 | issue=2 | date=June 26, 2019 | issn=2296-3529 | doi=10.1159/000500259 | pages=163–169| pmid=31700858 | pmc=6827451 }}</ref>
== Diagnosis ==
Primary cutaneous anaplastic large cell lymphoma is characterized histologically by large, irregular polygonal cells distributed in sheets throughout the [[dermis]] and occasionally the subcutis.<ref name="Paulli Berti Rosso Boveri 1995 pp. 1343–1354">{{cite journal | last1=Paulli | first1=M | last2=Berti | first2=E | last3=Rosso | first3=R | last4=Boveri | first4=E | last5=Kindl | first5=S | last6=Klersy | first6=C | last7=Lazzarino | first7=M | last8=Borroni | first8=G | last9=Menestrina | first9=F | last10=Santucci | first10=M | title=CD30/Ki-1-positive lymphoproliferative disorders of the skin--clinicopathologic correlation and statistical analysis of 86 cases: a multicentric study from the European Organization for Research and Treatment of Cancer Cutaneous Lymphoma Project Group. | journal=Journal of Clinical Oncology | publisher=American Society of Clinical Oncology (ASCO) | volume=13 | issue=6 | year=1995 | issn=0732-183X | doi=10.1200/jco.1995.13.6.1343 | pages=1343–1354| pmid=7751878 }}</ref>
== Treatment ==
Complete surgical resection and/or [[radiation therapy]] are used to treat primary cutaneous anaplastic large cell lymphoma as a single lesion; the majority of patients experience total remission.<ref name="Clinical, Morphological, Immunohistochemical, and Molecular Features">{{cite journal | last1=Ortiz-Hidalgo | first1=Carlos | last2=Pina-Oviedo | first2=Sergio | title=Primary Cutaneous Anaplastic Large Cell Lymphoma—A Review of Clinical, Morphological, Immunohistochemical, and Molecular Features | journal=Cancers | publisher=MDPI AG | volume=15 | issue=16 | date=August 14, 2023 | issn=2072-6694 | doi=10.3390/cancers15164098 | doi-access=free | page=4098| pmid=37627126 | pmc=10452173 }}</ref> While radiation of the primary lesion and the surrounding lymph nodes has been advised for patients whose local [[Lymph node|lymph nodes]] are involved, adding radiation to surgical excision does not seem to improve localized disease.<ref name="Liu Hoppe Kohler Harvell 2003 pp. 1049–1058">{{cite journal | last1=Liu | first1=Howard L. | last2=Hoppe | first2=Richard T. | last3=Kohler | first3=Sabine | last4=Harvell | first4=Jeff D. | last5=Reddy | first5=Sunil | last6=Kim | first6=Youn H. | title=Cd30+ cutaneous lymphoproliferative disorders: The stanford experience in lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma | journal=Journal of the American Academy of Dermatology | publisher=Elsevier BV | volume=49 | issue=6 | year=2003 | issn=0190-9622 | doi=10.1016/s0190-9622(03)02484-8 | pages=1049–1058| pmid=14639383 }}</ref>
== See also ==
* [[Lymphomatoid papulosis]]
* [[List of cutaneous conditions]]
== References ==
{{reflist}}
== Further reading ==
* {{cite journal | last1=Kumar | first1=Shimareet | last2=Pittaluga | first2=Stefania | last3=Raffeld | first3=Mark | last4=Guerrera | first4=Michael | last5=Seibel | first5=Nita L. | last6=Jaffe | first6=Elaine S. | title=Primary Cutaneous CD30-Positive Anaplastic Large Cell Lymphoma in Childhood: Report of 4 Cases and Review of the Literature | journal=Pediatric and Developmental Pathology | publisher=SAGE Publications | volume=8 | issue=1 | year=2005 | issn=1093-5266 | doi=10.1007/s10024-004-8087-6 | pages=52–60 | pmid=15719203 | s2cid=25798596 | ref=none}}
* {{cite journal | last1=Sarfraz | first1=H. | last2=Gentille | first2=C. | last3=Ensor | first3=J. | last4=Wang | first4=L. | last5=Wong | first5=S. | last6=Ketcham | first6=M. S. | last7=Joshi | first7=J. | last8=Pingali | first8=S. R. K. | title=Primary cutaneous anaplastic large-cell lymphoma: a review of the SEER database from 2005 to 2016 | journal=Clinical and Experimental Dermatology | publisher=Oxford University Press (OUP) | volume=46 | issue=8 | date=July 17, 2021 | issn=0307-6938 | doi=10.1111/ced.14777 | pages=1420–1426 | pmid=34081802 | s2cid=235335222 | ref=none}}
== External links ==
* [https://www.uptodate.com/contents/primary-cutaneous-anaplastic-large-cell-lymphoma UpToDate]
* [https://dermnetnz.org/topics/primary-cutaneous-anaplastic-large-cell-lymphoma DermNet]
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| ICD11           = {{ICD11|2B03.0}}
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[[Category:Lymphoid-related cutaneous conditions]]
[[Category:Non-Hodgkin lymphoma]]