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Review
. 2005 Jul;53(1):158-63.
doi: 10.1016/j.jaad.2005.01.133.

Mycosis fungoides with a CD56+ immunophenotype

E Mary Wain  1 , Guy E Orchard, Susan Mayou, David J Atherton, Klaus J Misch, Robin Russell-Jones
Affiliations
Review

Mycosis fungoides with a CD56+ immunophenotype

E Mary Wain et al. J Am Acad Dermatol. 2005 Jul.

Abstract

We report 3 cases of mycosis fungoides (MF) with a CD56+ cytotoxic immunophenotype. Each patient presented with a different clinical phenotype: one exhibited limited poikilodermatous patches (skin stage T1); one, widespread hypopigmented lesions (skin stage T2); and one, poikiloderma with a single cutaneous tumor (skin stage T3). MF was confirmed both histologically and by the presence of a T-cell receptor clone in lesional skin in all cases. CD56 and T-cell intracellular antigen-1 were expressed by the malignant lymphocytes in all patients and two expressed CD8. No sample demonstrated loss of the pan T-cell markers CD2 or CD3. None of the 3 developed systemic disease and T-cell receptor gene analysis of peripheral blood was polyclonal in all cases. Only 3 cases of CD56+ MF have been reported previously, none of which exhibited tumor-stage disease. Currently, the disease in our patients appears to be behaving in a manner similar to that predicted for MF with a normal immunophenotype but the prognosis has to be guarded in view of the rarity of this subtype.

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