피부 T세포 림프종

피부 T세포 림프종 (CTCL) 은 면역체계에 발생하는 암이며, 비호지킨 림프종의 일종입니다. 보통의 비호지킨 림프종(일반적으로 B세포 관련임)과 달리, CTCL 는 T세포의 돌연변이로 인해 발생합니다. 신체에서 암을 일으킬 수 있는 T세포는 처음에 피부로 이동한 후, 다양한 병변을 일으키는 원인이 됩니다. 이러한 병변은 질병이 진행되면서 모양이 바뀌는데, 일반적으로 아주 간지러울 수 있는 발진으로 시작해서 신체의 다른 부위로 퍼지기 전에, 결국 플라크와 종양을 형성합니다.

신호와 증상

이 종류들만 있는 것은 아니지만, 가장 흔한 유형인 균상식육종인지 세자리증후군인지에 따라 증상에 차이가 있을 수 있습니다. 앞에서 이야기한 유형들의 증상으로는 림프절 비대, 간 및 비장 비대, 비특이성 피부염 등이 있습니다.

원인

피부 T세포 림프종의 원인은 밝혀지지 않았습니다.

진단

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.

분류

Cutaneous T-cell lymphoma may be divided into the several subtypes.^:727–740 Mycosis fungoides is the most common form of CTCL and is responsible for half of all cases. A WHO-EORTC classification has been developed.

• 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

치료

Romidepsin

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:

• (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. 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.

역학

Of all cancers involving lymphocytes, 2% of cases are cutaneous T cell lymphomas. 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.

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.

참고

• Cutaneous B-cell lymphoma
• List of cutaneous conditions

External links

분류	[[d: |D]]
외부 자원

* DermNet dermal-infiltrative/cutaneous-t-cell-lymphoma

This article "피부 T세포 림프종" is from Wikipedia. The list of its authors can be seen in its historical and/or the page Edithistory:피부 T세포 림프종. Articles copied from Draft Namespace on Wikipedia could be seen on the Draft Namespace of Wikipedia and not main one.

---

 Facebook Page

🐤 Twitter Follow us on Twitter !

Read or create/edit this page in another language

• 피부 T세포 림프종 in English