Abstract:
To investigate the clinical, pathological, and immunophenotypic characteristics of subcutaneous panniculitis-like T cell lymphoma and to investigate the corresponding effective treatment and prognostic factors. Methods: The clinical, histologic, immunophenotypic features and treatments of 6 cases were described in detail. Results: All 6 patients presented with subcutaneous nodules and/or erythematous plaques with or without lymphadenopathy, and 2 cases had bone marrow involvement, 2 cases were positive for Epstein-Barr virus, and 5 cases had fever. All 6 patients presented with typical histologic changes. Intermediate to large-sized lymphocytes infiltrated preferentially into the lobular area of the subcutaneous tissue and displayed adipotropism with rimming of lymphocytes around adipocytes and empty lipid vacuoles with hyperchromatic nuclei. T cell markers were positive, B cell markers were negative; CD4 and CD8 expression were different. The cytotoxic proteins in some cases were positive. The three patients treated with CHOP all relapsed. Chemotherapy with gemcitabine for newly diagnosed and 4 relapsed patients achieved complete remission ( CR ) in 3 cases, partial remission ( PR ) in 1 case with no severe hematologic toxicity. One case with hemophagocytic syndrome ( HPS ) that was Epstein-Barr virus-positive died. Conclusion: Chemotherapy with gemcitabine is an effective regimen in the treatment of newly diagnosed and relapsed SPTCL patients. The presence of HPS and Epstein-Barr virus are the poor prognostic factors.