Primary cutaneous T-cell lymphomas (CTCL), rep resenting a heterogeneous gr
oup of non-Hodgkin's lymphomas (NHL), can be defined as clonal proliferatio
n of skin-infiltrating T lymphocytes primarily presenting in the cutaneous
compartment. They show a considerable variation in clinical presentation, h
istology, immunophenotype, and prognosis, which is best reflected by the pr
oposal of the Cutaneous Lymphoma Study Group of the European Organization f
or Research and Treatment of Cancer (EORTC). Due to the heterogeneity of CT
CL and the lack of curative therapy regimens, multiple strategies have been
proposed for the management of the different CTCL entities. this includes
topical application of corticosteroids, nitrogen mustard or carmustine (BCN
U), radiotherapy. including total skin electron beam irradiation, photo(che
mo)therapy, biological response modifiers, cytostatic chemotherapy, and com
bined regimens. More recently, fusion proteins and peptide vaccines have be
en introduced in the management of CTCL. Classification, staging, and treat
ment modalities are discussed in detail and summarized in a stage-adapted t
herapy regimen for CTCL. Copyright (C) 2000 by W.B. Saunders Company.