Treatment of rare cutaneous T-cell lymphoma and blastic plasmacytoid dendritic cell neoplasm

摘要

Among the group of primary cutaneous lymphomas several subtypes have very low incidence rates. Based on the revision of the WHO classification for lymphoid neoplasms (2016), an overview of rare cutaneous T-cell lymphoma (CTCL) subtypes is given and therapeutic approaches are detailed. The prognosis of the different subtypes is highly variable underlining the importance of adequate stage and subtype adapted treatment. In cases of indolent subtypes topical treatment, e.g. topical corticosteroids or UV phototherapy are often sufficient. For aggressive variants, early discussion of more aggressive systemic treatment options is warranted.