Mycosis fungoides classification: Difference between revisions

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*Primary [[cutaneous]] [[peripheral T cell lymphoma]], [[rare]] subtypes
*Primary [[cutaneous]] [[peripheral T cell lymphoma]], [[rare]] subtypes
**Primary [[cutaneous]] [[Gamma-delta T-cell antigen receptor|gamma-delta T cell lymphoma]]
**Primary [[cutaneous]] [[Gamma-delta T-cell antigen receptor|gamma-delta T cell lymphoma]]
**Primary [[cutaneous]] aggressive epidermotropic CD8+ T cell lymphoma (provisional)
**Primary [[cutaneous]] aggressive epidermotropic [[CD8+ T cells|CD8+ T cell]] [[Lymphomas|lymphoma]] (provisional)
**Primary cutaneous CD4+ small/medium-sized pleomorphic T cell lymphoproliferative disorder (provisional)
**Primary [[cutaneous]] CD4+ small/medium-sized pleomorphic T cell [[Lymphoproliferative disorders|lymphoproliferative disorder]] (provisional)
**Primary cutaneous acral CD8+ T cell lymphoma (provisional)
**Primary [[cutaneous]] acral [[CD8+ T cells|CD8+ T cell]] lymphoma (provisional)
*Primary cutaneous peripheral T cell lymphoma, not otherwise specified
*Primary cutaneous peripheral T cell lymphoma, not otherwise specified



Revision as of 20:06, 20 December 2018

Cutaneous T cell lymphoma Microchapters

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Patient Information

Overview

Classification

Mycosis fungoides
Sezary syndrome

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sogand Goudarzi, MD [2]

Overview

There are 3 classification methods used to classify cutaneous T cell lymphoma into several subtypes. Cutaneous T cell lymphoma may be classified into several subtypes based on WHO-EORTC classification.

Classification

According to world Health Organization (WHO) and European Organization for Research and Treatment of Cancer (EORTC) classification, cutaneous T cell and NK cell lymphomas may be classified into the following types:[1][2]


Cutaneous T cell lymphoma classification[3]
Name Description
Mycosis fungoides (MF)
  • Most common form of cutaneous T cell lymphoma
  • Starts in the skin in areas of the body not usually exposed to the sun
  • May appear as a scaly, pink patches on the skin
  • Signs can progress to the development of skin tumors in more advanced cases
Sézary syndrome (SS)
  • Often the more aggressive form of cutaneous T cell lymphoma
  • An advanced form of mycosis fungoides
  • Affects skin, blood, and lymph nodes
  • Large areas of the skin are affected
  • Skin is often red, itchy, painful, and peeling
  • Skin tumors can also appear
  • Entire body can be affected, also known as "red man syndrome"
Cutaneous T cell lymphoma classification[4]
Name Description
Primary or cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma
  • Characterized by localized or disseminated eruptive papules, nodules with tumors showing central ulceration, and necrosis or by superficial hyperkeratotic patches and plaques
  • Dissemination to other visceral sites (lung, testis, CNS, and oral mucosa)
  • Lymph nodes are seldom affected
  • Aggressive clinical course with median survival rate of 32 months
Primary cutaneous CD4-positive small/medium T-cell lymphoma
  • Clinical presentation is usually a solitary plaque or nodule, commonly on the face, neck, or upper trunk
  • The involvement of lower extremities is rare
  • Cutaneous patches are generally absent

References

  1. Matutes, E. (2018). "The 2017 WHO update on mature T- and natural killer (NK) cell neoplasms". International Journal of Laboratory Hematology. 40: 97–103. doi:10.1111/ijlh.12817. ISSN 1751-5521.
  2. Sundram, Uma (2018). "Cutaneous Lymphoproliferative Disorders". Advances In Anatomic Pathology: 1. doi:10.1097/PAP.0000000000000208. ISSN 1072-4109.
  3. Cutaneous T cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/cutaneous-t-cell-lymphoma/?region=on Accessed on January 19, 2016
  4. Cutaneous T cell lymphoma. Surveillance, Epidemiology, and End Results . http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52f7/ Accessed on January 19, 2016


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