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Mycosis fungoides with a CD56+ immunophenotype
E Mary Wain1, Guy E Orchard, Susan Mayou
1Skin Tumor Unit, St John's Institute of Dermatology, St Thomas' Hospital, London, UK. marywain@doctors.net.uk
Journal of the American Academy of Dermatology
|June 21, 2005
Summary
This study details three cases of mycosis fungoides (MF) with a rare CD56+ cytotoxic immunophenotype, presenting diverse clinical stages from patches to tumors. These findings expand understanding of MF variants and their potential clinical behavior.
Area of Science:
- Dermatology
- Immunology
- Oncology
Background:
- Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma.
- MF typically presents with a specific immunophenotype, but rare variants exist.
Observation:
- Three patients with mycosis fungoides (MF) exhibited a CD56+ cytotoxic immunophenotype.
- Clinical presentations varied, including limited patches (T1), widespread hypopigmented lesions (T2), and a cutaneous tumor (T3).
- Malignant lymphocytes expressed CD56, T-cell intracellular antigen-1, and in two cases, CD8, without loss of pan T-cell markers.
Findings:
- Histological confirmation and T-cell receptor (TCR) clonality in lesional skin supported MF diagnosis in all cases.
- No patients developed systemic disease, and peripheral blood TCR gene analysis remained polyclonal.
- This study adds to the limited reports of CD56+ MF, notably including a tumor-stage case.
Implications:
- The findings contribute to the characterization of rare CD56+ MF subtypes.
- While current behavior mirrors typical MF, the guarded prognosis for this rare variant warrants further investigation.
- Understanding this immunophenotypic variation is crucial for accurate diagnosis and management of cutaneous T-cell lymphomas.