Background: Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon type of skin lymphoma. The natural history, optimal treatment strategy, and prognostic factors associated with this malignancy are not well defined.
Methods: The authors performed a systematic analysis of all patients with SPTCL reported on in the English-language medical literature, with emphasis on specific clinical features, experiences involving the use of radiotherapy and systemic agents, and prognostic factors predictive of treatment response and clinical outcome.
Results: One hundred fifty-six patients with SPTCL were identified in the literature. Hemophagocytic syndrome (HPS) was a presenting feature in 37% of patients, and > 90% of patients required treatment at diagnosis. Prednisone was used frequently as initial therapy in patients who had less aggressive disease at presentation; however, durable complete remissions (CR) were infrequent. Anthracycline-based chemotherapy regimens were the most commonly used and most effective systemic treatment options, producing long-term CR in approximately 30% of patients. Among patients who received high-dose chemotherapy and stem cell transplantation (HDT-SCT) for refractory or recurrent disease, 92% achieved CR, with a median response duration of > or = 14 months. The presence of HPS at diagnosis and expression of the gamma/delta T-cell receptor (TCR) by tumor cells were associated with poor survival, whereas age was not. After a median follow-up of 24 months, 48% of patients died of disease. The median survival duration was 27 months.
Conclusions: SPTCL has an aggressive natural history. Nonetheless, a subgroup of patients with SPTCL can have long-term disease remission following anthracycline-based initial therapy or subsequent HDT-SCT. HPS and the TCR phenotype may be useful prognostic markers for patients with this malignancy.
Copyright 2004 American Cancer Society.