P107 Clinical, immunophenotypical and molecular analysis of a series of patients with poikilodermatous mycosis fungoides

Abstract

Introduction Poikilodermatous mycosis fungoides (PFM) is an uncommon variant of MF, characterized by poikilodermia, atrophy, mottled dyspigmentation and telangectases. Aim To analyze cases of PMF diagnosed at the lymphoma clinic of Andreas Sygros Hospital, in relation to their clinical presentation, immunophenotypic and molecular characteristics and disease course. Methods All patients with PFM were retrospectively evaluated. Patients with predominantly poikilodermatous lesions of MF were selected. Information on clinical, histological, immunohistochemical and molecular data were recorded, in addition to information on treatment and follow up. Results Eleven patients (4 males and 7 females) with PMF were identified. The mean age at diagnosis was 33.5 years (range 10–75 years). Ten out of 11 patients had early stage disease (IB) and one patient presented with tumors accompanying widespread poikilodermia (stage IIB). Two patients had associated lesions typical of lymphomatoid papulosis (LyP). In 45.5% of patients (5 of 11), the atypical lymphocytes were predominantly CD4-positive, whereas the same proportion of patients (5 of 11) had a cytotoxic phenotype. Finally, aberrant CD4-CD8- phenotype was found in one patient, while in seven patients there was a loss of CD7 antigen. PCR analysis disclosed the presence of clonal rearrangements of the genes of TcRγ receptor in 8 out of 11 skin biopsies. Following diagnosis, 8 patients were treated with PUVA. Of these, six showed complete resolution of skin lesions, one partial remission and one progressive disease. Two patients showed stable disease after UBV-NB and acitretin 35 mg/day respectively. Finally, patient with tumor stage MF underwent electron beam radiotherapy on tumors followed by PUVA plus interferon a-2b 5 MU tiw, with disease progression after 5 months of treatment. Conclusion Our data suggest that PMF is commonly diagnosed at a young age and can be frequently accompanied by LyP lesions. A cytotoxic immunophenotype is also more common in PMF. Patients with PMF respond well to phototherapy and may have a good overall prognosis.