Dual CD4/CD8-positive Ichthyosiform Mycosis Fungoides with Lymph Node, Peripheral Blood and Cardiac Involvement: A Case Report.
2016
Ichthyosiform mycosis fungoides (MF) is a rare variant of MF, occurring in 3.5% of MF patients according to one study (1, 2). Patients with ichthyosiform MF exhibit clinical manifestations similar to those of acquired ichthyosis; histologically, however, they show epidermotropism of atypical lymphocytes. Ichthyosiform MF is characterized by indolent course and usually good response to phototherapy. Even among patients with conventional MF, dual CD4/CD8-positive MF is extremely rare, in contrast to a relatively high incidence of a mixture of CD4-positive neoplastic cells and inflammatory CD8positive infiltrating lymphocytes. We report here a case of ichthyosiform MF with aggressive extracutaneous involvement, including cardiac involvement, with a dual CD4/CD8-positive immunophenotype.
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