Subcutaneous panniculitis-like T-cell lymphoma in a patient with long-term remission with standard chemotherapy.

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a very rare postthymic T-cell non-Hodgkin's lymphoma with poor prognosis. There is not a standard treatment for this disease. Here we describe the first case of SPTL with unusual periorbital involvement, pancytopenia, hepatic dysfunction and coagulopathy, which was successfully treated with a chemotherapy regimen of cyclophosphamide, hydroxydaunomycin (doxorubicin), Oncovin (vincristine) and prednisone (CHOP). Our case demonstrates that although the natural history of SPTL is aggressive, patients may respond effectively to combination chemotherapy. Early recognition of the classic subcutaneous lesions and its associated systemic signs, such as unusual periorbital involvement, liver dysfunction and hemophagocytic syndrome, is very important in managing this aggressive lymphoma. Immunohistochemical and genetic studies are helpful in confirming the diagnosis. Early initiation of aggressive chemotherapy is recommended for better clinical outcome.