Treatment of Subcutaneous Panniculitis-Like T-Cell Lymphoma

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is localized to subcutaneous tissue and can be associated with hemophagocytic lymphohistiocytosis (HLH).[1-4] Anecdotal reports suggest that the presence or absence of HLH is an important prognostic indicator.[5] Patients with SPTCL have cells that express alpha-beta phenotype.

Patients with gamma-delta phenotype have a more aggressive clinical course that is instead classified as primary cutaneous gamma-delta T-cell lymphoma.[6-8] For more information, see Peripheral T-Cell Non-Hodgkin Lymphoma Treatment.

The management of SPTCL depends on the clinical presentation—including the severity of symptoms, cytopenias, and the presence or absence of HLH—and the apparent disease trajectory and aggressiveness. Indolent or smoldering forms of SPTCL are often treated with immunosuppression, including oral methotrexate [9] or cyclosporine.[10] In contrast, aggressive forms are frequently treated with combination chemotherapy such as CHO(E)P (cyclophosphamide, doxorubicin, vincristine, and prednisone with or without etoposide) with variable responses per anecdotal reports.[11] The JAK2 inhibitor ruxolitinib has also been used in SPTCL with associated HLH, including anecdotal reports of responses in patients with disease that did not respond to CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) therapy.[12] In cases of particularly aggressive or relapsed disease, consolidation with allogeneic stem cell transplant has also been used.[13,14]