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Keywords: Mycosis fungoides, Ichthyosiform, Sezary syndrome, Anaplastic, CD3/TCR-
Anahtar Sözcükler: Mikozis fungoides, İktiyotik, Sezary sendromu, Anaplastik, CD3/TCR
To the Editor,
A 49-year-old male was admitted to our department with a 3-year history of pruritus and severe xerosis. Dermatological examination revealed squamation and ichthyosis with dark lamella involving the trunk and extremities (Figure 1). Physical examination revealed bilateral lower extremity edema.
Histopathological examination of the ichthyotic skin showed perivascular dermal lymphoid cell infiltration in the superficial dermis and nodular and dense lymphoid cellular infiltration in the deep dermis. Lymphoid cells were CD3+ and CD20- with a loss of CD7 expression. There were numerous (>25%) large, anaplastic cells with CD30 positivity among the lymphoid infiltrate. Clonal expansion of T cells in the lesional skin was demonstrated.
Complete blood count revealed 61.5x109/L (reference range: 4.511) leukocytes with 15% Sezary cells. The axillary lymph node was consistent with N3 mycosis fungoides (MF) involvement. Thorax, abdominal, and pelvic computed tomography was normal. Bone marrow biopsy revealed involvement with positive clonality.
Flow cytometric analysis of the peripheral blood revealed the CD3/ TCR complex in only 10% of the T cells. The CD4/CD8 ratio was 23 among T cells lacking CD3 expression and 1.6 among CD3+ cells.
The patient met the international criteria for Sezary syndrome [(SS); stage IVB, T4N3M1B2] and was diagnosed with ichthyosiform MF with large cell transformation with atypical flow cytometric phenotype [1,2]. According to the 2014 National Comprehensive Cancer...