Clinical outcome of patients with subcutaneous panniculitis-like T-cell lymphoma

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare form of cytotoxic T-cell lymphoma. The objective of this study was to characterize the clinical presentation, treatment, and prognosis of patients with SPTCL. Twenty-one patients with SPTCL were seen at Mayo Clinic (Rochester, Minnesota, USA) between July 1973 and June 2004. The median age at diagnosis was 42 years (range 23-80 years) and 15 (71%) were women. Constitutional symptoms occurred in 14 (67%) patients, including fever, serositis, arthralgias and myalgias. The Eastern Cooperative Oncology Group performance score was poor (3-4) in 3 (15%) patients. Liver enzymes (at least 2 enzymes, Aspartate aminotransferase (AST), alkaline phosphatase and/or lactate dehydrogenase) were elevated in 11 (52%) patients. Therapy consisted of chemotherapy in 13 (62%) patients, or other therapeutic interventions in 8 (38%) patients, including surgical excision, corticosteroids alone or in combination with either plaquenil, colchicine, hydroxychoroquine, or azathioprine. Bone marrow transplantation was performed in 5 (24%) patients, 3 autologous and 2 allogeneic. The median overall survival from diagnosis was 15 months (range 0.1-104 months). Two groups of patients were identified and categorized as having a favorable or unfavorable disease course. The factors associated with an unfavorable disease course were a low white blood cell count or elevated lactate dehydrogenase. Patients treated aggressively with stem cell transplantation appeared to have an improved overall survival.