Mycosis fungoides classification

Cutaneous T cell lymphoma Microchapters
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Overview
Classification Mycosis fungoides Sezary syndrome
Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2],Sogand Goudarzi, MD [3]

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:

• Mycosis fungoides
• Mycosis fungoides variants and subtypes
  • Folliculotropic mycosis fungoides
  • Pagetoid reticulosis
  • Granulomatous slack skin
• Sezary syndrome
• Adult T cell leukemia/lymphoma
• Primary cutaneous CD30+ lymphoproliferative disorders
  • Primary cutaneous anaplastic large cell lymphoma
  • Lymphomatoid papulosis
• Subcutaneous panniculitis-like T cell lymphoma
• Extranodal NK/T cell lymphoma, nasal type
• Primary cutaneous peripheral T cell lymphoma, rare subtypes
  • Primary cutaneous gamma-delta T cell lymphoma
  • Primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma (provisional)
  • Primary cutaneous CD4+ small/medium-sized pleomorphic T cell lymphoproliferative disorder (provisional)
  • Primary cutaneous acral CD8+ T cell lymphoma (provisional)
• Primary cutaneous peripheral T cell lymphoma, not otherwise specified

Cutaneous T cell lymphoma classification

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

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

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