Cutaneous T-Cell
Lymphoma (CTCL)
Mycosis Fungoides
Epidemiology:
Common sites:
- trunk
- extremities
- face
- scalp
Gross features:
- mycosis fungoides:
- eczema-like in
early stage
- scaly, red-brown
patches
- raised, scaling plaques
(may be confused with psoriasis)
- fungating nodules
- may ulcerate
- multiple, large,
red-brown nodules correlates with systemic spreading
- erythroderma may occur with seeding of the blood
(Sezary syndrome)
- diffuse
erythema and scaling of entire body surface
Histologic features:
- Sezary-Lutner cells (hallmark):
- T-helper cells
(CD4+) in a bandlike aggregate within the
superficial dermis
- Invade the
epidermis as single cells and small clusters (Pautrier microabscesses)
- Markedly infolded
nuclear membranes (cerebriform)
Immunophenotype:
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Marker:
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Sensitivity:
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Specificity:
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CD4
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- Aberrant
cell-surface antigens
Molecular features:
- clonal T-cell receptor gene rearrangements
Other features:
- spectrum of lymphoproliferative disorders affecting the skin
- mycosis fungoides
- eruptive nodular
type
- adult T-cell
leukemia/lymphoma type
- arises primarily
in the skin and may evolve to generalized lymphoma
- most remain
localized to skin for many years
References: