Systemic corticosteroids for subcutaneous panniculitis-like T-cell lymphoma

• Published in: British journal of dermatology (1951) Vol. 171; no. 4; pp. 891 - 894
• Main Authors: Guenova, E., Schanz, S., Hoetzenecker, W., DeSimone, J.A., Mehra, T., Voykov, B., Urosevic-Maiwald, M., Berneburg, M., Dummer, R., French, L.E., Kerl, K., Kamarashev, J., Fierlbeck, G., Cozzio, A.
• Format: Journal Article
• Language: English
• Published: Oxford Blackwell Publishing Ltd 01-10-2014; Wiley-Blackwell; Oxford University Press
• Subjects: Adult; Aged; Antineoplastic Agents, Hormonal - therapeutic use; Biological and medical sciences; Corticosteroids; Cross-Sectional Studies; Dermatology; Female; Hematologic and hematopoietic diseases; Humans; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma; Lymphoma, T-Cell - drug therapy; Male; Medical sciences; Middle Aged; Panniculitis - drug therapy; Patients; Phenotypes; Prednisolone - therapeutic use; Prognosis; Remission; Retrospective Studies; Skin involvement in other diseases. Miscellaneous. General aspects; Skin Neoplasms - drug therapy; T-cell lymphoma; Treatment Outcome; Corticosteroid; Skin disease; Dermatology; Subcutaneous; Panniculitis; Malignant hemopathy; Systemic; Non Hodgkin lymphoma; Immunological investigation; Lymphoproliferative syndrome; T-Lymphocyte; Adipose tissue disorders; Disseminated; Cancer
• ISSN: 0007-0963; 1365-2133
• DOI: 10.1111/bjd.13053

Summary
Background
Primary cutaneous γ/δ T‐cell lymphoma (PCGD‐TCL) is aggressive and has a poor prognosis. In contrast, subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) of the α/β 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.
Objectives
To assess the overall clinical response to systemic corticosteroids in the treatment of SPTCL.
Methods
This was a retrospective cross‐sectional study based on a patient data repository from two tertiary care university hospitals in Zürich (Switzerland) and Tübingen (Germany). The repository spanned 13 years.
Results
In four of the five patients (80%) with SPTCL, treatment with systemic corticosteroids induced a complete remission.
Conclusions
Systemic corticosteroids may be an excellent first‐line single‐agent therapy for SPTCL.
What's already known about this topic?
In the past, subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) and primary cutaneous γ/δ T‐cell lymphoma were not recognized as separate entities, and were both treated with aggressive polychemotherapy.
What does this study add?
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.