Subcutaneous panniculitislike T-cell lymphoma with hemophagocytosis: complete remission with BFM-90 protocol.

SUMMARY: Subcutaneous panniculitislike T-cell lymphoma (SPTCL) is an uncommon type of cutaneous lymphoma. In many cases, SPTCL is accompanied by hemophagocytic syndrome (HPS), resulting in prominent systemic symptoms. The natural history, optimal treatment strategy, and prognostic factors associated with this malignancy are not well defined. We report an 11-year-old boy of SPTCL with HPS who was initially treated with conventional cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy, but progressed later on therapy. Subsequently, the child was treated with multiagent combination chemotherapy as per BFM-90 protocol and achieved complete remission, and has remained so for 3 years. This report suggests the value of this particular multiagent combination chemotherapy regimen in the treatment of patients with SPTCL and HPS.