Zusammenfassung
BackgroundPrimary cutaneous / T-cell lymphoma (PCGD-TCL) is aggressive and has a poor prognosis. In contrast, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) of the / T-cell receptor phenotype is known to follow an indolent course and have a more favourable prognosis. In the past, PCGD-TCL and SPTCL were often considered to be a manifestation of the same disease, and aggressive systemic ...
Zusammenfassung
BackgroundPrimary cutaneous / T-cell lymphoma (PCGD-TCL) is aggressive and has a poor prognosis. In contrast, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) of the / T-cell receptor phenotype is known to follow an indolent course and have a more favourable prognosis. In the past, PCGD-TCL and SPTCL were often considered to be a manifestation of the same disease, and aggressive systemic polychemotherapy has commonly been the first-line therapy for both. Given the understanding that SPTCL is a separate and less aggressive entity, clinical data exclusively evaluating the efficacy of conservative treatment in SPTCL are needed. ObjectivesTo assess the overall clinical response to systemic corticosteroids in the treatment of SPTCL. MethodsThis was a retrospective cross-sectional study based on a patient data repository from two tertiary care university hospitals in Zurich (Switzerland) and Tubingen (Germany). The repository spanned 13years. ResultsIn four of the five patients (80%) with SPTCL, treatment with systemic corticosteroids induced a complete remission. ConclusionsSystemic corticosteroids may be an excellent first-line single-agent therapy for SPTCL. What's already known about this topic? <list list-type="bulleted" id="bjd13053-list-0001"> In the past, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) and primary cutaneous / T-cell lymphoma were not recognized as separate entities, and were both treated with aggressive polychemotherapy. What does this study add? <list list-type="bulleted" id="bjd13053-list-0002"> With new understanding, SPTCL is now known to be a separate and less aggressive disease, and aggressive treatment approaches should not be considered as a first-line treatment option. Systemic corticosteroids induce long-term remission and should be considered as the first-line treatment.
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