View source for HAEM5:Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: Primary cutaneous anaplastic large cell lymphoma

{{DISPLAYTITLE:Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: Primary cutaneous anaplastic large cell lymphoma}}
[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (WHO Classification, 5th ed.)]]

{{Under Construction}}

<blockquote class='blockedit'>{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Primary Cutaneous Anaplastic Large Cell Lymphoma]].
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<span style="color:#0070C0">(General Instructions – The main focus of these pages is the clinically significant genetic alterations in each disease type. Use [https://www.genenames.org/ <u>HUGO-approved gene names and symbols</u>] (italicized when appropriate), [https://varnomen.hgvs.org/ HGVS-based nomenclature for variants], as well as generic names of drugs and testing platforms or assays if applicable. Please complete tables whenever possible and do not delete them (add N/A if not applicable in the table and delete the examples); to add (or move) a row or column to a table, click within the table and select the > symbol that appears to be given options. Please do not delete or alter the section headings. The use of bullet points alongside short blocks of text rather than only large paragraphs is encouraged. Additional instructions below in italicized blue text should not be included in the final page content. Please also see </span><u>[[Author_Instructions]]</u><span style="color:#0070C0"> and [[Frequently Asked Questions (FAQs)|<u>FAQs</u>]] as well as contact your [[Leadership|<u>Associate Editor</u>]] or [mailto:CCGA@cancergenomics.org <u>Technical Support</u>])</span>

==Primary Author(s)*==

Theresa Spivey, MD, Shashirekha Shetty, PhD

__TOC__

==Cancer Category / Type==

*Mature T- and NK-cell Neoplasms

==Cancer Sub-Classification / Subtype==

*[[Primary Cutaneous CD30-positive T-cell lymphoproliferative disorders|Primary Cutaneous CD30-positive T-cell lymphoproliferative disorders (CD30+ LPDs)]]

==Definition / Description of Disease==


*Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a form of cutaneous T-cell lymphoma with majority (>75%) of tumor cells expressing CD30.
*CD30+ LPDs are a part of a spectrum of diseases with overlapping features and are associated with an excellent prognosis. The group includes lymphomatoid papulosis (LyP) and C-ALCL, which are distinguished by clinical features and disease course.
*C-ALCL is a distinct entity from systemic anaplastic large cell lymphoma (ALCL), which can have cutaneous involvement.
*Diagnosis must exclude large cell transformation of mycosis fungoides, which can express CD30.
*


<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}<ref name=":1">Arber DA, et al., (2017). Primary Cutaneous CD30-positive T-cell Lymphoproliferative Disorders, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Revised 4th edition. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Arber DA, Hasserjian RP, Le Beau MM, Orazi A, and Siebert R, Editors. IARC Press: Lyon, France, p392-396.</ref><ref name=":2" /><ref name=":3">{{Cite journal|last=Willemze|first=Rein|last2=Cerroni|first2=Lorenzo|last3=Kempf|first3=Werner|last4=Berti|first4=Emilio|last5=Facchetti|first5=Fabio|last6=Swerdlow|first6=Steven H.|last7=Jaffe|first7=Elaine S.|date=2019-04-18|title=The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas|url=https://doi.org/10.1182/blood-2018-11-881268|journal=Blood|volume=133|issue=16|pages=1703–1714|doi=10.1182/blood-2018-11-881268|issn=0006-4971}}</ref></blockquote>
==Synonyms / Terminology==

*Primary Cutaneous CD30-positive T-cell lymphoproliferative disorder

==Epidemiology / Prevalence==


*Second most common type of cutaneous T-cell lymphoma
*Median age: 60 years. Cases have been reported in children.
*Male:Female 2-3:1
*Disease-specific 5-year survival rate 95%


<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}<ref name=":1" /><ref name=":3" /></blockquote>
==Clinical Features==

Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table. Do not delete table.'') </span>
{| class="wikitable"
|'''Signs and Symptoms'''
|<span class="blue-text">EXAMPLE:</span> Asymptomatic (incidental finding on complete blood counts)

<span class="blue-text">EXAMPLE:</span> B-symptoms (weight loss, fever, night sweats)

<span class="blue-text">EXAMPLE:</span> Fatigue

<span class="blue-text">EXAMPLE:</span> Lymphadenopathy (uncommon)
|-
|'''Laboratory Findings'''
|<span class="blue-text">EXAMPLE:</span> Cytopenias

<span class="blue-text">EXAMPLE:</span> Lymphocytosis (low level)
|}


<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Features|The content below was from the old template. Please incorporate above.}}


*Solitary or localized nodules or tumors, with frequent ulceration. Multifocal lesions are seen in 20% of patients.
*Lesions may regress, either partially or completely, but cutaneous relapse is common.
*Extracutaneous dissemination is seen in 10-15% of cases, most cases involving regional lymph nodes.
*Associated with excellent prognosis, even in those with regional lymph node involvement or multifocal skin lesions.
*Anaplastic morphology does not impact disease course or prognosis.
*Distinguishing from LyP is important for therapy, as surgical excision and radiotherapy are first-line treatments in patients with C-ALCL.


<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}<ref name=":1" /><ref name=":2" /><ref name=":3" /></blockquote>
</blockquote>
==Sites of Involvement==


*Limited to cutaneous sites, most frequently affecting the trunk, face, and extremities.


<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}<ref name=":1" /></blockquote>
==Morphologic Features==


*Sheets or nodular dermal infiltrate of large pleomorphic, anaplastic, or immunoblastic cells
*Irregularly shaped nuclei
*Abundant, pale to eosinophilic, cytoplasm
*Reactive lymphocytes in the periphery
*Ulcerated lesions may resemble lymphomatoid papulosis with increased inflammatory infiltrate composed of T-cells, histiocytes, eosinophils, and neutrophils, with fewer CD30+ cells.

*In cases harboring ''DUSP22-IRF4'' translocations, CD30+ cells commonly display biphasic morphology - small, cerebriform lymphocytes within the epidermis and large transformed cells in dermis.


<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}<ref name=":1" /><ref name=":2" /><ref name=":3" /></blockquote>
==Immunophenotype==


{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (activated T-cell markers)*||CD4, CD30 (diagnosis requires >75% expression of tumor cells)
|-
|Variable (pan T-cell markers)||CD2, CD3, CD5
|-
|Positive||Cytotoxic proteins<sup>†</sup>: Granzyme B, TIA1, perforin

CD15 (40%), IRF4/MUM1, CLA (cutaneous lymphocyte antigen)
|-
|Negative
|EMA<sup>‡</sup>, ALK<sup>‡</sup>, PAX5, EBV, CD56 (rare cases with coexpression)
|}
<nowiki>*</nowiki>Some cases may have a T-cell phenotype of CD4-/CD8+ or CD4+/CD8+ or null-cell phenotype.

<sup>†</sup>Cases with DUSP22-IRF4 rearrangement tend to express 

<sup>‡</sup>EMA and ALK are typically positive in systemic ALCL, however there are rare cases of ALK+ primary C-ALCL.


<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}<ref name=":1" /><ref name=":2">{{Cite journal|last=Kempf|first=Werner|last2=Pfaltz|first2=Katrin|last3=Vermeer|first3=Maarten H.|last4=Cozzio|first4=Antonio|last5=Ortiz-Romero|first5=Pablo L.|last6=Bagot|first6=Martine|last7=Olsen|first7=Elise|last8=Kim|first8=Youn H.|last9=Dummer|first9=Reinhard|date=2011-10-13|title=EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma*|url=https://ashpublications.org/blood/article/118/15/4024/29010/EORTC-ISCL-and-USCLC-consensus-recommendations-for|journal=Blood|language=en|volume=118|issue=15|pages=4024–4035|doi=10.1182/blood-2011-05-351346|issn=0006-4971|pmc=PMC3204726|pmid=21841159}}</ref></blockquote>
==Chromosomal Rearrangements (Gene Fusions)==

Put your text here and fill in the table

{| class="wikitable sortable"
|-
!Chromosomal Rearrangement!!Genes in Fusion (5’ or 3’ Segments)!!Pathogenic Derivative!!Prevalence
!Diagnostic Significance (Yes, No or Unknown)
!Prognostic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
!Notes
|-
|<span class="blue-text">EXAMPLE:</span> t(9;22)(q34;q11.2)||<span class="blue-text">EXAMPLE:</span> 3'ABL1 / 5'BCR||<span class="blue-text">EXAMPLE:</span> der(22)||<span class="blue-text">EXAMPLE:</span> 20% (COSMIC)
<span class="blue-text">EXAMPLE:</span> 30% (add reference)
|Yes
|No
|Yes
|<span class="blue-text">EXAMPLE:</span>

The t(9;22) is diagnostic of CML in the appropriate morphology and clinical context (add reference). This fusion is responsive to targeted therapy such as Imatinib (Gleevec) (add reference).
|}		
			

<blockquote class='blockedit'>{{Box-round|title=v4:Chromosomal Rearrangements (Gene Fusions)|The content below was from the old template. Please incorporate above.}}

*Rearrangements of ''DUSP22-IRF4'' locus on chromosome 6p25.3 are found in 25% of primary C-ALCL and in rare cases of LyP and transformed mycosis fungoides. The rearrangements have not found in systemic ALCL or other T-cell LPDs.<ref>{{Cite journal|last=Wada|first=David A.|last2=Law|first2=Mark E.|last3=Hsi|first3=Eric D.|last4=Dicaudo|first4=David J.|last5=Ma|first5=Linglei|last6=Lim|first6=Megan S.|last7=Souza|first7=Aieska de|last8=Comfere|first8=Nneka I.|last9=Weenig|first9=Roger H.|date=2011-04|title=Specificity of IRF4 translocations for primary cutaneous anaplastic large cell lymphoma: a multicenter study of 204 skin biopsies|url=https://pubmed.ncbi.nlm.nih.gov/21169992|journal=Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc|volume=24|issue=4|pages=596–605|doi=10.1038/modpathol.2010.225|issn=1530-0285|pmc=3122134|pmid=21169992}}</ref>
*''TYK2''  (19p13) rearrangements in 15% of CD30+ lymphoproliferative disorders, with recurrent ''NPM1-TYK2'' fusions reported in primary C-ALCL and LyP.<ref name=":4">{{Cite journal|last=Velusamy|first=Thirunavukkarasu|last2=Kiel|first2=Mark J.|last3=Sahasrabuddhe|first3=Anagh A.|last4=Rolland|first4=Delphine|last5=Dixon|first5=Catherine A.|last6=Bailey|first6=Nathanael G.|last7=Betz|first7=Bryan L.|last8=Brown|first8=Noah A.|last9=Hristov|first9=Alexandra C.|date=2014-12-11|title=A novel recurrent NPM1-TYK2 gene fusion in cutaneous CD30-positive lymphoproliferative disorders|url=https://doi.org/10.1182/blood-2014-07-588434|journal=Blood|volume=124|issue=25|pages=3768–3771|doi=10.1182/blood-2014-07-588434|issn=0006-4971}}</ref>
*''TP63'' rearrangements on 3q28 are rare in C-ALCL.<ref name=":0">{{Cite journal|last=Pedersen|first=Martin Bjerregård|last2=Hamilton-Dutoit|first2=Stephen Jacques|last3=Bendix|first3=Knud|last4=Ketterling|first4=Rhett P.|last5=Bedroske|first5=Patrick P.|last6=Luoma|first6=Ivy M.|last7=Sattler|first7=Christopher A.|last8=Boddicker|first8=Rebecca L.|last9=Bennani|first9=N. Nora|date=2017-07-27|title=DUSP22 and TP63 rearrangements predict outcome of ALK-negative anaplastic large cell lymphoma: a Danish cohort study|url=https://doi.org/10.1182/blood-2016-12-755496|journal=Blood|volume=130|issue=4|pages=554–557|doi=10.1182/blood-2016-12-755496|issn=0006-4971|pmc=PMC5533203|pmid=28522440}}</ref>
*Rare cases of primary C-ALCL contain ''ALK'' rearrangements and associated expression of ALK.<ref>{{Cite journal|last=Melchers|first=Rutger C.|last2=Willemze|first2=Rein|last3=van de Loo|first3=Merel|last4=van Doorn|first4=Remco|last5=Jansen|first5=Patty M.|last6=Cleven|first6=Arjen H. G.|last7=Solleveld|first7=Nienke|last8=Bekkenk|first8=Marcel W.|last9=van Kester|first9=Marloes S.|date=2020-06|title=Clinical, Histologic, and Molecular Characteristics of Anaplastic Lymphoma Kinase-positive Primary Cutaneous Anaplastic Large Cell Lymphoma|url=https://journals.lww.com/ajsp/Abstract/2020/06000/Clinical,_Histologic,_and_Molecular.8.aspx|journal=The American Journal of Surgical Pathology|language=en-US|volume=44|issue=6|pages=776–781|doi=10.1097/PAS.0000000000001449|issn=0147-5185}}</ref>
*

</blockquote>


<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Significance (Diagnosis, Prognosis and Therapeutic Implications).|Please incorporate this section into the relevant tables found in:
* Chromosomal Rearrangements (Gene Fusions)
* Individual Region Genomic Gain/Loss/LOH
* Characteristic Chromosomal Patterns
* Gene Mutations (SNV/INDEL)}}

*''DUSP22-IRF4'' rearrangement have not been shown to impact disease behavior prognosis.<ref>{{Cite journal|last=Willemze|first=Rein|last2=Cerroni|first2=Lorenzo|last3=Kempf|first3=Werner|last4=Berti|first4=Emilio|last5=Facchetti|first5=Fabio|last6=Swerdlow|first6=Steven H.|last7=Jaffe|first7=Elaine S.|date=2019-04-18|title=The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas|url=https://ashpublications.org/blood/article/133/16/1703/260505/The-2018-update-of-the-WHOEORTC-classification-for|journal=Blood|language=en|volume=133|issue=16|pages=1703–1714|doi=10.1182/blood-2018-11-881268|issn=0006-4971|pmc=PMC6473500|pmid=30635287}}</ref>
*''TP63'' rearrangements are associated with refractoriness to chemotherapy and poor prognosis<ref name=":0" />
*

</blockquote>
==Individual Region Genomic Gain / Loss / LOH==

Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Includes aberrations not involving gene fusions. Can include references in the table. Can refer to CGC workgroup tables as linked on the homepage if applicable. Do not delete table.'') </span>

{| class="wikitable sortable"
|-
!Chr #!!Gain / Loss / Amp / LOH!!Minimal Region Genomic Coordinates [Genome Build]!!Minimal Region Cytoband
!Diagnostic Significance (Yes, No or Unknown)
!Prognostic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
!Notes
|-
|<span class="blue-text">EXAMPLE:</span>

7
|<span class="blue-text">EXAMPLE:</span> Loss
|<span class="blue-text">EXAMPLE:</span>

chr7:1- 159,335,973 [hg38]
|<span class="blue-text">EXAMPLE:</span>

chr7
|Yes
|Yes
|No
|<span class="blue-text">EXAMPLE:</span>

Presence of monosomy 7 (or 7q deletion) is sufficient for a diagnosis of AML with MDS-related changes when there is ≥20% blasts and no prior therapy (add reference).  Monosomy 7/7q deletion is associated with a poor prognosis in AML (add reference).
|-
|<span class="blue-text">EXAMPLE:</span>

8
|<span class="blue-text">EXAMPLE:</span> Gain
|<span class="blue-text">EXAMPLE:</span>

chr8:1-145,138,636 [hg38]
|<span class="blue-text">EXAMPLE:</span>

chr8
|No
|No
|No
|<span class="blue-text">EXAMPLE:</span>

Common recurrent secondary finding for t(8;21) (add reference).
|}

<blockquote class='blockedit'>{{Box-round|title=v4:Genomic Gain/Loss/LOH|The content below was from the old template. Please incorporate above.}}


{| class="wikitable sortable"
|-
!Chromosome Number!!Gain/Loss/Amp/LOH
!Associated Genes
|-
|7q31||Gain
|''MET''
|-
|6q16-6q21||Loss
|''PRDM1''
|-
|13q34
|Loss
|''CDC16/CUL4A''
|}

*The genomic gains and losses listed above are the most common and seen in 45% of cases.
*


<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}<ref>{{Cite journal|last=van Kester|first=Marloes S.|last2=Tensen|first2=Cornelis P.|last3=Vermeer|first3=Maarten H.|last4=Dijkman|first4=Remco|last5=Mulder|first5=Aat A.|last6=Szuhai|first6=Karoly|last7=Willemze|first7=Rein|last8=van Doorn|first8=Remco|date=2010-02|title=Cutaneous Anaplastic Large Cell Lymphoma and Peripheral T-Cell Lymphoma NOS Show Distinct Chromosomal Alterations and Differential Expression of Chemokine Receptors and Apoptosis Regulators|url=http://dx.doi.org/10.1038/jid.2009.270|journal=Journal of Investigative Dermatology|volume=130|issue=2|pages=563–575|doi=10.1038/jid.2009.270|issn=0022-202X}}</ref></blockquote>
</blockquote>
==Characteristic Chromosomal Patterns==

Put your text here <span style="color:#0070C0">(''EXAMPLE PATTERNS: hyperdiploid; gain of odd number chromosomes including typically chromosome 1, 3, 5, 7, 11, and 17; co-deletion of 1p and 19q; complex karyotypes without characteristic genetic findings; chromothripsis. Do not delete table.'')</span>

{| class="wikitable sortable"
|-
!Chromosomal Pattern
!Diagnostic Significance (Yes, No or Unknown)
!Prognostic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
!Notes
|-
|<span class="blue-text">EXAMPLE:</span>

Co-deletion of 1p and 18q
|Yes
|No
|No
|<span class="blue-text">EXAMPLE:</span>

See chromosomal rearrangements table as this pattern is due to an unbalanced derivative translocation associated with oligodendroglioma (add reference).
|}

<blockquote class='blockedit'>{{Box-round|title=v4:Characteristic Chromosomal Aberrations / Patterns|The content below was from the old template. Please incorporate above.}}

*Clonal T-cell receptor gene rearrangement is detected in majority of cases. <ref>{{Cite journal|last=Greisser|first=Johannes|last2=Palmedo|first2=Gabriele|last3=Sander|first3=Christian|last4=Kutzner|first4=Heinz|last5=Kazakov|first5=Dmitry V.|last6=Roos|first6=Malgorzata|last7=Burg|first7=Günter|last8=Kempf|first8=Werner|date=2006-11|title=Detection of clonal rearrangement of T-cell receptor genes in the diagnosis of primary cutaneous CD30 lymphoproliferative disorders|url=https://pubmed.ncbi.nlm.nih.gov/17083688|journal=Journal of Cutaneous Pathology|volume=33|issue=11|pages=711–715|doi=10.1111/j.1600-0560.2006.00560.x|issn=0303-6987|pmid=17083688}}</ref>

</blockquote>
==Gene Mutations (SNV / INDEL)==

Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: This table is not meant to be an exhaustive list; please include only genes/alterations that are recurrent and common as well as either disease defining and/or clinically significant. Can include references in the table. For clinical significance, denote associations with FDA-approved therapy (not an extensive list of applicable drugs) and NCCN or other national guidelines if applicable. Can also refer to CGC workgroup tables as linked on the homepage if applicable as well as any high impact papers or reviews of gene mutations in this entity. Do not delete table.'') </span>

{| class="wikitable sortable"
|-
!Gene; Genetic Alteration!!'''Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other)'''!!'''Prevalence (COSMIC /  TCGA / Other)'''!!'''Concomitant Mutations'''!!'''Mutually Exclusive Mutations'''
!'''Diagnostic Significance (Yes, No or Unknown)'''
!Prognostic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
!Notes
|-
|<span class="blue-text">EXAMPLE:</span> TP53; Variable LOF mutations

<span class="blue-text">EXAMPLE:</span>

EGFR; Exon 20 mutations

<span class="blue-text">EXAMPLE:</span> BRAF; Activating mutations
|<span class="blue-text">EXAMPLE:</span> TSG
|<span class="blue-text">EXAMPLE:</span> 20% (COSMIC)

<span class="blue-text">EXAMPLE:</span> 30% (add Reference)
|<span class="blue-text">EXAMPLE:</span> IDH1 R123H
|<span class="blue-text">EXAMPLE:</span> EGFR amplification
|
|
|
|<span class="blue-text">EXAMPLE:</span>  Excludes hairy cell leukemia (HCL) (add reference).
<br />
|}
Note: A more extensive list of mutations can be found in cBioportal (https://www.cbioportal.org/), COSMIC (https://cancer.sanger.ac.uk/cosmic), ICGC (https://dcc.icgc.org/) and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.


<blockquote class='blockedit'>{{Box-round|title=v4:Gene Mutations (SNV/INDEL)|The content below was from the old template. Please incorporate above.}}
N/A

</blockquote>
==Epigenomic Alterations==

N/A

==Genes and Main Pathways Involved==

Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Can include references in the table. Do not delete table.'')</span>
{| class="wikitable sortable"
|-
!Gene; Genetic Alteration!!Pathway!!Pathophysiologic Outcome
|-
|<span class="blue-text">EXAMPLE:</span> BRAF and MAP2K1; Activating mutations
|<span class="blue-text">EXAMPLE:</span> MAPK signaling
|<span class="blue-text">EXAMPLE:</span> Increased cell growth and proliferation
|-
|<span class="blue-text">EXAMPLE:</span> CDKN2A; Inactivating mutations
|<span class="blue-text">EXAMPLE:</span> Cell cycle regulation
|<span class="blue-text">EXAMPLE:</span> Unregulated cell division
|-
|<span class="blue-text">EXAMPLE:</span>  KMT2C and ARID1A; Inactivating mutations
|<span class="blue-text">EXAMPLE:</span>  Histone modification, chromatin remodeling
|<span class="blue-text">EXAMPLE:</span>  Abnormal gene expression program
|}

<blockquote class='blockedit'>{{Box-round|title=v4:Genes and Main Pathways Involved|The content below was from the old template. Please incorporate above.}}

*''DUSP22'' rearrangements associated with decreased production in the enzyme, dual-specificity phosphatase-22, which regulates MAPK signaling pathway.<ref name=":0" />
*''NPM1-TYK2'' fusion is associated with constitutive STAT signaling.<ref name=":4" />

</blockquote>
==Genetic Diagnostic Testing Methods==


*Requires clinical and histopathologic correlation to diagnose and separate from lymphomatoid papulosis and large cell transformation of mycosis fungoides.
*Must exclude systemic involvement by ALCL with cutaneous involvement or history of mycosis fungoides.


<blockquote class='blockedit'>{{Box-round|title=Unassigned References|The following referenees were placed in the header. Please place them into the appropriate locations in the text.}}<ref name=":1" /></blockquote>
==Familial Forms==

N/A

==Additional Information==

N/A

==Links==

*[[HAEM4:Primary Cutaneous CD30 Positive T-cell Lymphoproliferative Disorders]]
*[[HAEM5:Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: Lymphomatoid papulosis]]
*[[HAEM5:ALK-negative anaplastic large cell lymphoma]]
*[[HAEM5:ALK-positive anaplastic large cell lymphoma]]
*[[HAEM5:Mycosis fungoides]]

==References==
(use the "Cite" icon at the top of the page) <span style="color:#0070C0">(''Instructions: Add each reference into the text above by clicking on where you want to insert the reference, selecting the “Cite” icon at the top of the page, and using the “Automatic” tab option to search such as by PMID to select the reference to insert. The reference list in this section will be automatically generated and sorted.''</span> <span style="color:#0070C0">''If a PMID is not available, such as for a book, please use the “Cite” icon, select “Manual” and then “Basic Form”, and include the entire reference''</span><span style="color:#0070C0">''.''</span><span style="color:#0070C0">) </span> <references />

'''

==Notes==
<nowiki>*</nowiki>Primary authors will typically be those that initially create and complete the content of a page.  If a subsequent user modifies the content and feels the effort put forth is of high enough significance to warrant listing in the authorship section, please contact the CCGA coordinators (contact information provided on the homepage).  Additional global feedback or concerns are also welcome.
<nowiki>*</nowiki>''Citation of this Page'': “Primary cutaneous CD30-positive T-cell lymphoproliferative disorder: Primary cutaneous anaplastic large cell lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Primary_cutaneous_CD30-positive_T-cell_lymphoproliferative_disorder:_Primary_cutaneous_anaplastic_large_cell_lymphoma</nowiki>.
[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases P]]