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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/28 14:07:59」(JST)
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"CTCL" redirects here. For the book by Loren Pope, see Colleges That Change Lives.
| Cutaneous T cell lymphoma |
|
Micrograph showing cutaneous T-cell lymphoma. H&E stain.
|
| Classification and external resources |
| Specialty |
Hematology and oncology |
| ICD-10 |
C84.0, C84.1 |
| ICD-9 |
202.1, 202.2 |
| ICD-O |
M9700/3, M9701/3 |
| DiseasesDB |
8595 |
| eMedicine |
med/3486 |
| MeSH |
D016410 |
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's lymphomas (which are generally B cell related), CTCL is caused by a mutation of T cells. The malignant 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 metastasizing to other parts of the body.
Contents
- 1 Classification
- 2 Treatment
- 3 Epidemiology
- 4 See also
- 5 References
- 6 External links
Classification
Cutaneous T-cell lymphomas may be divided into the following types:[1]:727–740
-
- Mycosis fungoides
- 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
A WHO-EORTC classification has been developed.[2][3]
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 are:[4]
- (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
Other (off label) Treatments include:
- Topical and oral corticosteroids
- Bexarotene (Targretin) gel and capsules
- Carmustine (BCNU, a nitrosourea)
- Mechlorethamine (Nitrogen Mustard)
- Phototherapy (Broad & Narrow Band UVB or PUVA)
- Local & Total Skin Electron Beam Radiation
- 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)
Other drugs are under investigation (for example panobinostat and Resimmune).
In 2010, the U.S. Food and Drug Administration granted orphan drug designation for a topical treatment for pruritus in cutaneous T-cell lymphoma to a pharmaceutical company called Elorac.[5]
Epidemiology
Of all cancers involving the same class of blood cell, 2% of cases are cutaneous T cell lymphomas.[6]
There is some evidence of a relationship with human T-lymphotropic virus.[7]
See also
- Cutaneous B-cell lymphoma
- List of cutaneous conditions
References
- ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
- ^ 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. 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.
- ^ http://www.istockanalyst.com/article/viewnewspaged/articleid/3622028/pageid/1
- ^ Elorac, Inc. Announces Orphan Drug Designation for Novel Topical Treatment for Pruritus in Cutaneous T-cell Lymphoma (CTCL) website
- ^ Turgeon, Mary Louise (2005). Clinical hematology: theory and procedures. Hagerstown, MD: Lippincott Williams & Wilkins. p. 283. ISBN 0-7817-5007-5.
Frequency of lymphoid neoplasms. (Source: Modified from WHO Blue Book on Tumour of Hematopoietic and Lymphoid Tissues. 2001, p. 2001.)
- ^ Nicot C (March 2005). "Current views in HTLV-I-associated adult T-cell leukemia/lymphoma". Am. J. Hematol. 78 (3): 232–9. doi:10.1002/ajh.20307. PMID 15726602.
External links
- DermNet dermal-infiltrative/cutaneous-t-cell-lymphoma
- Information on cutaneous T-cell lymphomas from Lymphoma Information Network
- cutaneous T-cell lymphoma from H. Lee Moffitt Cancer Center & Research Institute
- The Cutaneous Lymphoma Foundation
- Skin Research Center lab Hopital St Louis, Paris (France) Dir. Dr. A. Bensussan
|
Hematological malignancy/leukemia histology (ICD-O 9590–9989, C81–C96, 200–208)
Lymphoid/Lymphoproliferative, Lymphomas/Lymphoid leukemias (9590–9739, 9800–9839)
|
|
B cell
(lymphoma,
leukemia)
(most CD19
|
By development/
marker |
| TdT+ |
- ALL (Precursor B acute lymphoblastic leukemia/lymphoma)
|
|
| CD5+ |
- mantle zone (Mantle cell)
|
|
| CD22+ |
- Prolymphocytic
- CD11c+ (Hairy cell leukemia)
|
|
| CD79a+ |
- germinal center/follicular B cell (Follicular
- Burkitt's
- GCB DLBCL
- Primary cutaneous follicular lymphoma)
- marginal zone/marginal-zone B cell (Splenic marginal zone
- MALT
- Nodal marginal zone
- Primary cutaneous marginal zone lymphoma)
|
|
| RS (CD15+, CD30+) |
- Classic Hodgkin's lymphoma (Nodular sclerosis)
- CD20+ (Nodular lymphocyte predominant Hodgkin's lymphoma)
|
|
PCDs/PP
(CD38+/CD138+) |
- see immunoproliferative immunoglobulin disorders
|
|
|
| By infection |
- KSHV (Primary effusion)
- EBV (Lymphomatoid granulomatosis
- Post-transplant lymphoproliferative disorder)
- HIV (AIDS-related lymphoma)
- Helicobacter pylori (MALT lymphoma)
|
|
| Cutaneous |
- Diffuse large B-cell lymphoma
- Intravascular large B-cell lymphoma
- Primary cutaneous marginal zone lymphoma
- Primary cutaneous immunocytoma
- Plasmacytoma
- Plasmacytosis
- Primary cutaneous follicular lymphoma
|
|
|
| T/NK |
T cell
(lymphoma,
leukemia)
(most CD3
|
By development/
marker |
- TdT+: ALL (Precursor T acute lymphoblastic leukemia/lymphoma)
- prolymphocyte (Prolymphocytic)
- CD30+ (Anaplastic large-cell lymphoma
- Lymphomatoid papulosis type A)
|
|
| Cutaneous |
| MF+variants |
- indolent: Mycosis fungoides
- Pagetoid reticulosis
- Granulomatous slack skin
aggressive: Sézary disease
- Adult T-cell leukemia/lymphoma
|
|
| Non-MF |
- CD30-: Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma
- Pleomorphic T-cell lymphoma
- Lymphomatoid papulosis type B
- CD30+: CD30+ cutaneous T-cell lymphoma
- Secondary cutaneous CD30+ large cell lymphoma
- Lymphomatoid papulosis type A
|
|
|
| Other peripheral |
- Hepatosplenic
- Angioimmunoblastic
- Enteropathy-associated T-cell lymphoma
- Peripheral T-cell lymphoma-Not-Otherwise-Specified (Lennert lymphoma)
- Subcutaneous T-cell lymphoma
|
|
| By infection |
- HTLV-1 (Adult T-cell leukemia/lymphoma)
|
|
|
NK cell/
(most CD56) |
- Aggressive NK-cell leukemia
- Blastic NK cell lymphoma
|
|
| T or NK |
- EBV (Extranodal NK-T-cell lymphoma/Angiocentric lymphoma)
- Large granular lymphocytic leukemia
|
|
|
| Lymphoid+myeloid |
- Acute biphenotypic leukaemia
|
|
| Lymphocytosis |
- Lymphoproliferative disorders (X-linked lymphoproliferative disease
- Autoimmune lymphoproliferative syndrome)
- Leukemoid reaction
- Diffuse infiltrative lymphocytosis syndrome
|
|
|
| Cutaneous lymphoid hyperplasia |
- Cutaneous lymphoid hyperplasia
- with bandlike and perivascular patterns
- with nodular pattern
- Jessner lymphocytic infiltrate of the skin
|
|
|
Index of the immune system
|
|
| Description |
- Physiology
- cells
- autoantigens
- autoantibodies
- complement
- surface antigens
- IG receptors
|
|
| Disease |
- Allergies
- Immunodeficiency
- Immunoproliferative immunoglobulin disorders
- Hypersensitivity and autoimmune disorders
- Neoplasms and cancer
|
|
| Treatment |
- Procedures
- Drugs
- antihistamines
- immunostimulants
- immunosuppressants
- monoclonal antibodies
|
|
|
UpToDate Contents
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English Journal
- Targeting histone deacetylases in T-cell lymphoma.
- Moskowitz AJ1, Horwitz SM1.
- Leukemia & lymphoma.Leuk Lymphoma.2017 Jun;58(6):1306-1319. doi: 10.1080/10428194.2016.1247956. Epub 2016 Nov 4.
- PMID 27813438
- Latest insights into pathogenesis of mycosis fungoides and cutaneous T-cell lymphoma.
- McGirt LY1.
- Giornale italiano di dermatologia e venereologia : organo ufficiale, Societa italiana di dermatologia e sifilografia.G Ital Dermatol Venereol.2017 Apr;152(2):158-168. doi: 10.23736/S0392-0488.16.05527-9. Epub 2016 Dec 16.
- PMID 27982549
- Brentuximab Beats Standard Therapies for CTCL.
- [No authors listed]
- Cancer discovery.Cancer Discov.2017 Mar;7(3):OF3. doi: 10.1158/2159-8290.CD-NB2017-012. Epub 2017 Jan 24.
- Compared with a physician's choice of either methotrexate or bexarotene, the CD30-directed antibody-drug conjugate brentuximab vedotin led to significantly superior clinical responses in patients with cutaneous T-cell lymphomas, according to the results of the international phase III ALCANZA trial.�
- PMID 28119332
Japanese Journal
- 希少疾患の治療とメディカルニーズ 希少がん治療の現状 : 皮膚T細胞リンパ腫
- 今月の症例 リンパ管細静脈吻合術後に急速に増悪したStewart-Treves症候群の1例
Related Links
- 概要 皮膚に生じる悪性リンパ腫の一群で、複数あるリンパ球のうち腫瘍の由来となる細胞がT細胞であるものをいいます。日本では皮膚に生じる悪性リンパ腫の約90％が皮膚T細胞性リンパ腫（CTCL）です。CTCLに含まれる病気には菌状息肉 ...
- To honor the 20th anniversary of the first edition of Loren Pope's classic book and the 10th anniversary of the nonprofit organization, Colleges That Change Lives has established a scholarship for students who enroll at a CTCL-member ...
- 伊藤忠テクノソリューションズ(CTC)のWebサイトです。CTCは、コンサルテーション・SIからアウトソーシングまで、お客様のITライフサイクルを支えるソリューションプロバイダです。 ... 藤田光里選手（プロゴルファー）応援サイト 藤田 ...
Related Pictures
★リンクテーブル★
[★]
- 英
- cutaneous T-cell lymphoma、CTCL
- 関
- 皮膚T細胞性リンパ腫
[★]
- 英
- cutaneous T-cell lymphoma、CTCL
- 関
- 皮膚T細胞リンパ腫
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