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: Sogand Goudarzi, MD [2]

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

• The symptoms of mycosis fungoides usually develop in the first nonspecific, indolent inflammatory such as : topic dermatitis, nonspecific chronic dermatitis, or parapsoriasis ( large-plaque parapsoriasis).
• second/ third decade of life, and start with symptoms such as ___.
• The symptoms of (disease name) typically develop ___ years after exposure to ___.
• If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
• Mycosis fungoides in early stage characterized with nonspecific dermatitis or consists patches observed on the lower trunk and buttocks.
• 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 (B)
B0	0 Absence of significant blood involvement: 5% of peripheral blood lymphocytes are atypical (Sezary) cells B0a Clone negative B0b Clone positive
B1	Low blood tumour burden: >5% of peripheral blood lymphocytes are atypical (Sezary) cells but does not meet the criteria of B2 B1a Clone negative B1b Clone positive
B2	High blood tumour burden: 1000/mL Sezary cells with positive clone

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

clinical stages
Stage	T	N	M	B	DDS
IA	1	0	0	0/1	98
IB	2	0	0	0/1	89
IIA	1.2	1.2	0	0/1	89
IIB	3	0-2	0	0/1	56
IIIA	4	0-2	0	0	54
IIIB	4	0-2	0	1	48
IVA1	1-4	0-2	0	2	41
IVA2	1-4	3	0	0-2	23
IVB	1-4	0-3	1	0-2	18

• [5-year disease free survival (DSS)]
• 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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