Mycosis fungoides natural history, complications and prognosis

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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

If left untreated, cutaneous T cell lymphoma may progress to develop cutaneous patches, plaque, and tumors. Depending on the extent of the lymphoma at the time of diagnosis, the prognosis may vary.

Natural History

• Mycosis fungoides is initially an indolent lymphoma that may later develop peripheral lymphadenopathy and can finally progress to widespread visceral involvement.
• Cutaneous T cell lymphoma is usually initially seen by dermatologists with patients presenting with skin lesions such as erythematous patches or plaque.
• Patients often have a history of several years of eczematous or dermatitic skin lesions before the diagnosis is finally established.
• The skin lesions then progress from the patch stage to the plaque stage to cutaneous tumors.

Complications

• Common complications of Cutaneous T cell lymphoma include:
  • Mycosis Fungoides increased risk of secondary malignancies such as primary malignancy, especially Hodgkin lymphoma, chronic leukemia, and lung cancer.
  • [Complication 2]
  • [Complication 3]

Prognosis

• Cutaneous T cell lymphoma is usually a slow-growing (indolent) lymphoma.
• The prognosis for people with cutaneous T cell lymphoma is based on the extent of disease and how the person responds to treatment.
• Although more advanced stages of cutaneous T cell lymphoma may not be cured, the lymphoma can still be controlled with treatment.

Favorable prognosis

• Early stage disease
• Lymphoma is confined to the skin

Unfavorable prognosis

• More advanced disease
• Lymphoma has spread to lymph nodes
• Lymphoma has spread to other organs

Staging

The staging of cutaneous T cell lymphoma is based on skin and lymph node involvement.

Staging for cutaneous T cell lymphoma(Mycosis fungoides (MF) and Sezary syndrome have same critera for staging) is provided in the following table:

Staging for mycosis fungoides and Sezary syndrome
Skin(T)
T1	Limited patches, papules, and/or plaques covering <10% of the skin surface. May further stratify into T1a (patch only) versus T1b (plaque patch)
T2	Patches, papules, or plaques covering 10% of the skin surface. May further stratify into T2a (patch only) versus T2b (plaque patch).
T3	One or more tumours (1-cm diameter)
T4	Confluence of erythema covering 80% body surface area
Node(N)
N0	No clinically abnormal peripheral lymph nodes; biopsy not required
N1	Clinically abnormal lymph nodes; histopathology Dutch grade 1 or NCI LN0-2
N1a	Clone negative
N1b	Clone posetive
N2	Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3
N2a	Clone negatove
N2b	Clone posetive
N3	Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3e4 or NCI LN4; clone positive or negative
NX	Clinically abnormal peripheral lymph nodes; no histologic confirmation
Visceral(M)
M0	No visceral organ involvement
M1	Visceral involvement (must have pathology confirmation and organ involved should be specified)
Blood
B0	0 Absence of significant blood involvement: �5% of peripheral blood lymphocytes are atypical (Se´zary) cells B0a Clone negative B0b Clone positive
B1	Low blood tumour burden: >5% of peripheral blood lymphocytes are atypical (Se´zary) cells but does not meet the criteria of B2 B1a Clone negative B1b Clone positive
B2	High blood tumour burden: 1000/mL Se´zary cells with positive clone

The staging of Sezary syndrome is based on the clinical stages:

Staging of cutaneous T cell lymphoma

Stage	Involvement
stage I
I A	Less than 10% of the skin is covered with patches or plaques
I B	10% or more of the skin is covered with patches or plaques
stage II
II A	Any amount of the skin is covered in patches or plaques Lymph nodes are enlarged, but do not contain cancer
II B	There is one or more raised tumors on the skin Lymph nodes may or may not be enlarged and do not contain cancer
stage III
III	Almost all of the skin is reddened There may or may not be patches, plaques or skin tumors Lymph nodes may or may not be enlarged and do not contain cancer
stage IV
IV A	Cancer has spread to the lymph nodes, but not to other organs in the body
IV B	Cancer has spread to other organs in the body, including the blood and bone marrow Lymph nodes may be enlarged and may contain cancer

References

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