Immunophenotypic and molecular features, clinical outcomes, treatments, and prognostic factors associated with subcutaneous panniculitis-like T-cell lymphoma: A systematic analysis of 156 patients reported in the literature

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 -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 a 14 months. The presence of HPS at diagnosis and expression of the γ/δ 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.