48 research outputs found
Mycosis fungoides and SĂ©zary syndrome: 2019 update on diagnosis, riskâstratification, and management
Disease OverviewCutaneous Tâcell lymphomas (CTCL) are a heterogenous group of Tâcell neoplasms involving the skin, the majority of which may be classified as Mycosis fungoides (MF) or SĂ©zary syndrome (SS).DiagnosisThe diagnosis of MF or SS requires the integration of clinical and histopathologic data.RiskâAdapted TherapyTNMB (tumor, node, metastasis, blood) staging remains the most important prognostic factor in MF/SS and forms the basis for a âriskâadapted,â multiâdisciplinary approach to treatment. For patients with disease limited to the skin, skinâdirected therapies are preferred, as both diseaseâspecific and overall survival for these patients is favorable. In contrast, patients with advancedâstage disease with significant nodal, visceral or blood involvement are generally approached with systemic therapies. These include biologicâresponse modifiers, histone deacetylase (HDAC) inhibitors, or antibodyâbased strategies, in an escalating fashion. In highlyâselected patients, allogeneic stemâcell transplantation may be considered, as this may be curative in some patients.Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/151292/1/ajh25577_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/151292/2/ajh25577.pd