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Cutaneous T-cell lymphoma

{{Short description|Type of immune system cancer}}

{{redirect|CTCL|the book by Loren Pope|Colleges That Change Lives}}

{{Infobox medical condition (new)

| name = Cutaneous T-cell lymphoma

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| image = Cutaneous T-cell lymphoma - very high mag.jpg

| caption = Micrograph showing cutaneous T-cell lymphoma. H&E stain

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| field = Hematology and oncology

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Cutaneous T-cell lymphoma (CTCL) is a class of non-Hodgkin lymphoma, which is a type of cancer of the immune system. Unlike most non-Hodgkin lymphomas (which are generally B-cell-related), CTCL is caused by a mutation of T cells. The cancerous T cells in the body initially migrate to the skin, causing various lesions to appear. These lesions change shape as the disease progresses, typically beginning as what appears to be a rash which can be very itchy and eventually forming plaques and tumors before spreading to other parts of the body.

Signs and symptoms

The presentation depends if it is mycosis fungoides or Sézary syndrome, the most common, though not the only types.

Among the symptoms for the aforementioned types are: enlarged lymph nodes, an enlarged liver and spleen, and non-specific dermatitis.{{Cite web|date=2016-06-02|title=Cutaneous T-Cell Lymphoma: Practice Essentials, Background, Pathophysiology|url=http://emedicine.medscape.com/article/2139720-overview}}

Cause

The cause of CTCL remains largely unknown, but several external risk factors have been proposed as potential triggers and promoters of the disease. These include the use of hydrochlorothiazide diuretics, therapy-induced immunosuppression, and possible infections by a range of viral (e.g., HTLV-1, HTLV-2, HIV, Epstein-Barr virus, Cytomegalovirus, HHV-6, HHV-7, HHV-8 (KSHV), and Polyomaviruses such as Merkel cell polyomavirus) and bacterial or fungal pathogens (including Staphylococcus aureus, Mycobacterium leprae, Chlamydophila pneumoniae, and dermatophytes). The level of evidence varies among the different factors.{{Cite journal |last1=Ghazawi |first1=Feras M. |last2=Alghazawi |first2=Nebras |last3=Le |first3=Michelle |last4=Netchiporouk |first4=Elena |last5=Glassman |first5=Steven J. |last6=Sasseville |first6=Denis |last7=Litvinov |first7=Ivan V. |date=2019 |title=Environmental and Other Extrinsic Risk Factors Contributing to the Pathogenesis of Cutaneous T Cell Lymphoma (CTCL) |journal=Frontiers in Oncology |volume=9 |page=300 |doi=10.3389/fonc.2019.00300 |doi-access=free |pmid=31106143 |pmc=6499168 |issn=2234-943X}}

Diagnosis

A point-based algorithm for the diagnosis for early forms of cutaneous T-cell lymphoma was proposed by the International Society for Cutaneous Lymphomas in 2005.{{Cite journal|last1=Pimpinelli|first1=Nicola|last2=Olsen|first2=Elise A.|last3=Santucci|first3=Marco|last4=Vonderheid|first4=Eric|last5=Haeffner|first5=Andreas C.|last6=Stevens|first6=Seth|last7=Burg|first7=Guenter|last8=Cerroni|first8=Lorenzo|last9=Dreno|first9=Brigitte|date=December 2005|title=Defining early mycosis fungoides|journal=Journal of the American Academy of Dermatology|volume=53|issue=6|pages=1053–1063|doi=10.1016/j.jaad.2005.08.057|pmid=16310068 |hdl=2158/311708|url=https://flore.unifi.it/bitstream/2158/311708/1/Defining%20early%20mycosis%20fungoides.pdf|hdl-access=free}}

= Classification =

Cutaneous T-cell lymphoma may be divided into the several subtypes.{{cite book |author1=James, William D. |author2=Berger, Timothy G. |title=Andrews' Diseases of the Skin: clinical Dermatology |publisher=Saunders Elsevier |year=2006 |isbn=978-0-7216-2921-6 |display-authors=etal}}{{rp|727–740}} Mycosis fungoides is the most common form of CTCL and is responsible for half of all cases.{{cite journal|last1=Sidiropoulos|first1=KG|last2=Martinez-Escala|first2=ME|last3=Yelamos|first3=O|last4=Guitart|first4=J|last5=Sidiropoulos|first5=M|title=Primary cutaneous T-cell lymphomas: a review|journal=Journal of Clinical Pathology|date=December 2015|volume=68|issue=12|pages=1003–10|doi=10.1136/jclinpath-2015-203133|pmid=26602417|s2cid=44407772|type=Review}} A WHO-EORTC classification has been developed.{{Cite journal | last1 = Willemze | first1 = R. | last2 = Jaffe | first2 = ES. | last3 = Burg | first3 = G. | last4 = Cerroni | first4 = L. | last5 = Berti | first5 = E. | last6 = Swerdlow | first6 = SH. | last7 = Ralfkiaer | first7 = E. | last8 = Chimenti | first8 = S. | last9 = Diaz-Perez | first9 = JL. | last10 = Duncan | first10 = L. M. | last11 = Grange | first11 = F | last12 = Harris | first12 = N. L. | last13 = Kempf | first13 = W | last14 = Kerl | first14 = H | last15 = Kurrer | first15 = M | last16 = Knobler | first16 = R | last17 = Pimpinelli | first17 = N | last18 = Sander | first18 = C | last19 = Santucci | first19 = M | last20 = Sterry | first20 = W | last21 = Vermeer | first21 = M. H. | last22 = Wechsler | first22 = J | last23 = Whittaker | first23 = S | last24 = Meijer | first24 = C. J. | title = WHO-EORTC classification for cutaneous lymphomas | journal = Blood | volume = 105 | issue = 10 | pages = 3768–85 |date=May 2005 | doi = 10.1182/blood-2004-09-3502 | pmid = 15692063 | display-authors = 8 | hdl = 2434/566817 | doi-access = free | hdl-access = free }}{{Cite journal | last1 = Khamaysi | first1 = Z. | last2 = Ben-Arieh | first2 = Y. | last3 = Izhak | first3 = OB. | last4 = Epelbaum | first4 = R. | last5 = Dann | first5 = EJ. | last6 = Bergman | first6 = R. | title = The applicability of the new WHO-EORTC classification of primary cutaneous lymphomas to a single referral center | journal = Am J Dermatopathol | volume = 30 | issue = 1 | pages = 37–44 |date=Feb 2008 | doi = 10.1097/DAD.0b013e31815f9841 | pmid = 18212543 | s2cid = 13571836 }}{{cite journal | vauthors = Melchers RC, Willemze R, van de Loo M, van Doorn R, Jansen PM, Cleven AH, Solleveld N, Bekkenk MW, van Kester MS, Diercks GF, Vermeer MH, Quint KD | title = Clinical, Histologic, and Molecular Characteristics of Anaplastic Lymphoma Kinase-positive Primary Cutaneous Anaplastic Large Cell Lymphoma | journal = The American Journal of Surgical Pathology | volume = 44 | issue = 6 | pages = 776–781 | date = June 2020 | pmid = 32412717 | doi = 10.1097/PAS.0000000000001449 | s2cid = 213450901 | url = https://research.rug.nl/en/publications/634ff7f7-9d94-4950-be22-c0f5bb48135a| hdl = 1887/3280088 | hdl-access = free }}{{cite journal | vauthors = Saleh JS, Subtil A, Hristov AC | title = Primary cutaneous T-cell lymphoma: a review of the most common entities with focus on recent updates | journal = Human Pathology | volume = 138 | issue = | pages = 76–102 | date = August 2023 | pmid = 37307932 | doi = 10.1016/j.humpath.2023.06.001 | url = }}

{{columns-list|colwidth=40em|

:* Pagetoid reticulosis

:* Sézary syndrome

:* Granulomatous slack skin

:* Lymphomatoid papulosis

:* Pityriasis lichenoides chronica

:* Pityriasis lichenoides et varioliformis acuta

:* CD30+ cutaneous T-cell lymphoma

:* Secondary cutaneous CD30+ large cell lymphoma

:* Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma

:* Pleomorphic T-cell lymphoma

:* Lennert lymphoma

:* Subcutaneous T-cell lymphoma

:* Angiocentric lymphoma

:* Blastic NK-cell lymphoma

:* Primary cutaneous anaplastic large-cell lymphoma

:* Primary cutaneous acral CD8 positive T cell lymphoproliferative disorder

}}

Treatment

File:Romidepsin ball and spoke.png

There is no cure for CTCL, but there are a variety of treatment options available and some CTCL patients are able to live normal lives with this cancer, although symptoms can be debilitating and painful, even in earlier stages. FDA approved treatments include the following:

Histone deacetylase (HDAC) inhibitors are shown to have antiproliferative and cytotoxic properties against CTCL.{{Cite book|title=Clinician's Guide to Mycosis Fungoides|last=Beigi|first=Pooya Khan Mohammad|date=2017|publisher=Springer International Publishing|isbn=9783319479064|pages=23–34|language=en|doi=10.1007/978-3-319-47907-1_6|chapter = Treatment}} Other (off label) treatments include:

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In 2010, the U.S. Food and Drug Administration granted orphan drug designation for naloxone lotion as a treatment for pruritus in cutaneous T-cell lymphoma to a pharmaceutical company called Elorac.[http://eon.businesswire.com/news/eon/20101130005333/en/Elorac-Announces-Orphan-Drug-Designation-Topical-Treatment Elorac, Inc. Announces Orphan Drug Designation for Novel Topical Treatment for Pruritus in Cutaneous T-cell Lymphoma (CTCL)] {{Webarchive|url=https://web.archive.org/web/20101230160054/http://eon.businesswire.com/news/eon/20101130005333/en/Elorac-Announces-Orphan-Drug-Designation-Topical-Treatment |date=2010-12-30 }} website

Epidemiology

Of all cancers involving lymphocytes, 2% of cases are cutaneous T cell lymphomas.{{cite book|author=Turgeon, Mary Louise|title=Clinical hematology: theory and procedures|url=https://archive.org/details/clinicalhematolo0004turg|url-access=registration|publisher=Lippincott Williams & Wilkins|location=Hagerstown, MD|year=2005|isbn=978-0-7817-5007-3|quote=Frequency of lymphoid neoplasms. (Source: Modified from WHO Blue Book on Tumour of Hematopoietic and Lymphoid Tissues. 2001, p. 2001.)|page=[https://archive.org/details/clinicalhematolo0004turg/page/n328 283]}} CTCL is more common in men and in African-American people. The incidence of CTCL in men is 1.6 times higher than in women.{{cite journal|last1=Devata|first1=S|last2=Wilcox|first2=RA|title=Cutaneous T-Cell Lymphoma: A Review with a Focus on Targeted Agents|journal=American Journal of Clinical Dermatology|date=June 2016|volume=17|issue=3|pages=225–37|doi=10.1007/s40257-016-0177-5|pmid=26923912|s2cid=28520647|type=Review}}

There is some evidence of a relationship with human T-lymphotropic virus (HTLV) with the adult T-cell leukemia/lymphoma subtype. No definitive link between any viral infection or environmental factor has been definitely shown with other CTCL subtypes.

See also

References

{{Reflist}}

External links

{{Medical resources

| ICD10 = {{ICD10|C|84|0|c|81}}, {{ICD10|C|84|1|c|81}}

| ICD9 = {{ICD9|202.1}}, {{ICD9|202.2}}

| ICDO = {{ICDO|9700|3}}, {{ICDO|9701|3}}

| OMIM =

| MedlinePlus =

| eMedicineSubj = med

| eMedicineTopic = 3486

| DiseasesDB = 8595

| MeshID = D016410

}}

  • {{DermNet|dermal-infiltrative/cutaneous-t-cell-lymphoma}}

{{Hematological malignancy histology}}

{{Medicine}}

{{DEFAULTSORT:Cutaneous T-Cell Lymphoma}}

Category:Lymphoid-related cutaneous conditions

Category:Lymphoma

Category:Rare cancers