Кожная Т-клеточная лимфома

Кожная Т-клеточная лимфома
Кожная Т-клеточная лимфома
	Микрофотография, показывающая кожную Т-клеточную лимфому. H & E Пятна
Специальность	Гематология и онкология

Кожная Т-клеточная лимфома ( CTCL ) представляет собой класс неходжкинской лимфомы , которая является типом рака иммунной системы . В отличие от большинства неходжкинских лимфом (которые, как правило, связаны с B-клетками ), CTCL вызван мутацией Т-клеток . Раковые Т -клетки в организме первоначально мигрируют на кожу различных поражений , вызывая появление . Эти поражения изменяют форму по мере развития болезни, как правило, начинается как то, что кажется сыпью , которая может быть очень зудящей и в конечном итоге образует бляшки и опухоли, прежде чем распространяться на другие части тела.

Признаки и симптомы

Презентация зависит от того, является ли это грибов микоза или синдрома Сезари , наиболее распространенными, но не единственными типами. Среди симптомов вышеупомянутых типов являются: увеличенные лимфатические узлы , увеличенная печень и селезенка , а также неспецифический дерматит . ^{[ 1 ]}

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.^[2]

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.^[3]

Classification

Cutaneous T-cell lymphoma may be divided into the several subtypes.^[4]^{: 727–740} Mycosis fungoides is the most common form of CTCL and is responsible for half of all cases.^[5] A WHO-EORTC classification has been developed.^[6]^[7]^[8]^[9]

Treatment

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:^[10]

(1999) Denileukin diftitox (Ontak)
(2000) Bexarotene (Targretin) a retinoid
(2006) Vorinostat (Zolinza) a hydroxymate histone deacetylase (HDAC) inhibitor
(2009) Romidepsin (Istodax) a cyclic peptide histone deacetylase (HDAC) inhibitor
(2018) Poteligeo (mogamulizumab-kpkc)

Histone deacetylase (HDAC) inhibitors are shown to have antiproliferative and cytotoxic properties against CTCL.^[11] Other (off label) treatments include:

Topical and oral corticosteroids
Bexarotene (Targretin) gel and capsules
Carmustine (BCNU, a nitrosourea)
Mechlorethamine (nitrogen mustard)
Phototherapy (broad and narrow band UVB or PUVA)
Electron therapy
Conventional radiation therapy
Photopheresis
Interferons
Alemtuzumab (Campath-1H)
Methotrexate
Pentostatin and other purine analogues (Fludarabine, 2-deoxychloroadenosine)
Liposomal doxorubicin (Doxil)
Gemcitabine (Gemzar)
Cyclophosphamide
Bone marrow / stem cells
Allogenic transplantation
Forodesine (inhibits purine nucleoside phosphorylase)

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.^[12]

Epidemiology

Of all cancers involving lymphocytes, 2% of cases are cutaneous T cell lymphomas.^[13] CTCL is more common in men and in African-American people.^[10] The incidence of CTCL in men is 1.6 times higher than in women.^[10]

There is some evidence of a relationship with human T-lymphotropic virus (HTLV) with the adult T-cell leukemia/lymphoma subtype.^[10] No definitive link between any viral infection or environmental factor has been definitely shown with other CTCL subtypes.^[10]

References

^ "Cutaneous T-Cell Lymphoma: Practice Essentials, Background, Pathophysiology". 2016-06-02.
^ Ghazawi, Feras M.; Alghazawi, Nebras; Le, Michelle; Netchiporouk, Elena; Glassman, Steven J.; Sasseville, Denis; Litvinov, Ivan V. (2019). "Environmental and Other Extrinsic Risk Factors Contributing to the Pathogenesis of Cutaneous T Cell Lymphoma (CTCL)". Frontiers in Oncology. 9: 300. doi:10.3389/fonc.2019.00300. ISSN 2234-943X. PMC 6499168. PMID 31106143.
^ Pimpinelli, Nicola; Olsen, Elise A.; Santucci, Marco; Vonderheid, Eric; Haeffner, Andreas C.; Stevens, Seth; Burg, Guenter; Cerroni, Lorenzo; Dreno, Brigitte (December 2005). "Defining early mycosis fungoides" (PDF). Journal of the American Academy of Dermatology. 53 (6): 1053–1063. doi:10.1016/j.jaad.2005.08.057. hdl:2158/311708. PMID 16310068.
^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 978-0-7216-2921-6.
^ Sidiropoulos, KG; Martinez-Escala, ME; Yelamos, O; Guitart, J; Sidiropoulos, M (December 2015). "Primary cutaneous T-cell lymphomas: a review". Journal of Clinical Pathology (Review). 68 (12): 1003–10. doi:10.1136/jclinpath-2015-203133. PMID 26602417. S2CID 44407772.
^ Willemze, R.; Jaffe, ES.; Burg, G.; Cerroni, L.; Berti, E.; Swerdlow, SH.; Ralfkiaer, E.; Chimenti, S.; et al. (May 2005). "WHO-EORTC classification for cutaneous lymphomas". Blood. 105 (10): 3768–85. doi:10.1182/blood-2004-09-3502. hdl:2434/566817. PMID 15692063.
^ Khamaysi, Z.; Ben-Arieh, Y.; Izhak, OB.; Epelbaum, R.; Dann, EJ.; Bergman, R. (Feb 2008). "The applicability of the new WHO-EORTC classification of primary cutaneous lymphomas to a single referral center". Am J Dermatopathol. 30 (1): 37–44. doi:10.1097/DAD.0b013e31815f9841. PMID 18212543. S2CID 13571836.
^ 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 (June 2020). "Clinical, Histologic, and Molecular Characteristics of Anaplastic Lymphoma Kinase-positive Primary Cutaneous Anaplastic Large Cell Lymphoma". The American Journal of Surgical Pathology. 44 (6): 776–781. doi:10.1097/PAS.0000000000001449. hdl:1887/3280088. PMID 32412717. S2CID 213450901.
^ Saleh JS, Subtil A, Hristov AC (August 2023). "Primary cutaneous T-cell lymphoma: a review of the most common entities with focus on recent updates". Human Pathology. 138: 76–102. doi:10.1016/j.humpath.2023.06.001. PMID 37307932.
^ Jump up to: ^a ^b ^c ^d ^e Devata, S; Wilcox, RA (June 2016). "Cutaneous T-Cell Lymphoma: A Review with a Focus on Targeted Agents". American Journal of Clinical Dermatology (Review). 17 (3): 225–37. doi:10.1007/s40257-016-0177-5. PMID 26923912. S2CID 28520647.
^ Beigi, Pooya Khan Mohammad (2017). "Treatment". Clinician's Guide to Mycosis Fungoides. Springer International Publishing. pp. 23–34. doi:10.1007/978-3-319-47907-1_6. ISBN 9783319479064.
^ Elorac, Inc. Announces Orphan Drug Designation for Novel Topical Treatment for Pruritus in Cutaneous T-cell Lymphoma (CTCL) Archived 2010-12-30 at the Wayback Machine website
^ Тургеон, Мэри Луиза (2005). Клиническая гематология: теория и процедуры . Hagerstown, MD: Lippincott Williams & Wilkins. п. 283 . ISBN 978-0-7817-5007-3 Полем Частота лимфоидных новообразований. (Источник: модифицирован из Blue Book о опухоли гематопоэтических и лимфоидных тканей. 2001, стр. 2001.)

Внешние ссылки

Дермнет -инфильтративная/кожная-клеточная лимфома

[1] "Cutaneous T-Cell Lymphoma: Practice Essentials, Background, Pathophysiology". 2016-06-02.

[2] Ghazawi, Feras M.; Alghazawi, Nebras; Le, Michelle; Netchiporouk, Elena; Glassman, Steven J.; Sasseville, Denis; Litvinov, Ivan V. (2019). "Environmental and Other Extrinsic Risk Factors Contributing to the Pathogenesis of Cutaneous T Cell Lymphoma (CTCL)". Frontiers in Oncology. 9: 300. doi:10.3389/fonc.2019.00300. ISSN 2234-943X. PMC 6499168. PMID 31106143.

[3] Pimpinelli, Nicola; Olsen, Elise A.; Santucci, Marco; Vonderheid, Eric; Haeffner, Andreas C.; Stevens, Seth; Burg, Guenter; Cerroni, Lorenzo; Dreno, Brigitte (December 2005). "Defining early mycosis fungoides" (PDF). Journal of the American Academy of Dermatology. 53 (6): 1053–1063. doi:10.1016/j.jaad.2005.08.057. hdl:2158/311708. PMID 16310068.

[Andrews-4] James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 978-0-7216-2921-6.

[Sidiropoulos2015-5] Sidiropoulos, KG; Martinez-Escala, ME; Yelamos, O; Guitart, J; Sidiropoulos, M (December 2015). "Primary cutaneous T-cell lymphomas: a review". Journal of Clinical Pathology (Review). 68 (12): 1003–10. doi:10.1136/jclinpath-2015-203133. PMID 26602417. S2CID 44407772.

[6] Willemze, R.; Jaffe, ES.; Burg, G.; Cerroni, L.; Berti, E.; Swerdlow, SH.; Ralfkiaer, E.; Chimenti, S.; et al. (May 2005). "WHO-EORTC classification for cutaneous lymphomas". Blood. 105 (10): 3768–85. doi:10.1182/blood-2004-09-3502. hdl:2434/566817. PMID 15692063.

[7] Khamaysi, Z.; Ben-Arieh, Y.; Izhak, OB.; Epelbaum, R.; Dann, EJ.; Bergman, R. (Feb 2008). "The applicability of the new WHO-EORTC classification of primary cutaneous lymphomas to a single referral center". Am J Dermatopathol. 30 (1): 37–44. doi:10.1097/DAD.0b013e31815f9841. PMID 18212543. S2CID 13571836.

[pmid32412717-8] 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 (June 2020). "Clinical, Histologic, and Molecular Characteristics of Anaplastic Lymphoma Kinase-positive Primary Cutaneous Anaplastic Large Cell Lymphoma". The American Journal of Surgical Pathology. 44 (6): 776–781. doi:10.1097/PAS.0000000000001449. hdl:1887/3280088. PMID 32412717. S2CID 213450901.

[pmid37307932-9] Saleh JS, Subtil A, Hristov AC (August 2023). "Primary cutaneous T-cell lymphoma: a review of the most common entities with focus on recent updates". Human Pathology. 138: 76–102. doi:10.1016/j.humpath.2023.06.001. PMID 37307932.

[Devata2016-10] Jump up to: ^a ^b ^c ^d ^e Devata, S; Wilcox, RA (June 2016). "Cutaneous T-Cell Lymphoma: A Review with a Focus on Targeted Agents". American Journal of Clinical Dermatology (Review). 17 (3): 225–37. doi:10.1007/s40257-016-0177-5. PMID 26923912. S2CID 28520647.

[11] Beigi, Pooya Khan Mohammad (2017). "Treatment". Clinician's Guide to Mycosis Fungoides. Springer International Publishing. pp. 23–34. doi:10.1007/978-3-319-47907-1_6. ISBN 9783319479064.

[CTCL-12] Elorac, Inc. Announces Orphan Drug Designation for Novel Topical Treatment for Pruritus in Cutaneous T-cell Lymphoma (CTCL) Archived 2010-12-30 at the Wayback Machine website

[isbn0-7817-5007-5-13] Тургеон, Мэри Луиза (2005). Клиническая гематология: теория и процедуры . Hagerstown, MD: Lippincott Williams & Wilkins. п. 283 . ISBN 978-0-7817-5007-3 Полем Частота лимфоидных новообразований. (Источник: модифицирован из Blue Book о опухоли гематопоэтических и лимфоидных тканей. 2001, стр. 2001.)

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Классификация	Дюймовый ICD - 10 : C84.0 , C84.1 ICD - 9 -cm : 202.1 , 202.2 ICD-O : M9700/3 , M9701/3 Сетка : D016410 DEDEASHESDB : 8595
Внешние ресурсы	Эмедицина : Med/3486