cutaneous T cell lymphoma


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cutaneous T cell lymphoma

Mycosis fungoides Oncology A heterogeneous group of relatively uncommon NHLs, which first appear on the skin Epidemiology ±1000 cases occur/yr, US, affecting ages 50-60; ♂:♀ ratio, 2:1; black:white, 2:1 Clinical Long–4-10 yrs Hx of eczematous rashes, which may evolve to parapsoriasis en plaque or poikiloderma atrophicans vasculare  Pathology Epidermotropism, paracortical proliferation in lymph nodes, periarterial proliferation in spleen; aggregates of epithelioid cells, prominent vascular channels, BM involvement Management Nitrogen mustard, BCNU, PUVA therapy, RT Response rate 80% if T1; 30–60% if T2; 20% if T3. See Redman syndrome, Sézary syndrome.
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The cutaneous T cell lymphomas (CTCL) include diverse malignancies: mycosis fungoides (MF), Sezary syndrome, lymphomatoid papulosis (LyP), anaplastic large-cell lymphoma (ALCL) and many less known varieties.