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]

Overview

There are 3 classification methods used to classify cutaneous T cell lymphoma into several subtypes.

Classification

Cutaneous T-cell lymphomas may be classified into the following types:

• 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

Based on the organ involvement, cutaneous T cell lymphoma may be classified into:

• Mycosis fungoides (MF)
• Sézary syndrome (SS)

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 or red rash or patches on the skin Signs can progress to skin tumours 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 tumours 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) possible Lymph nodes seldom affected Aggressive clinical course with median survival 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 Involvement of lower extremities is rare There should be an absence of patches

References

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