SUBCUTANEOUS T-CELL LYMPHOMA WITH ASSOCIATED HEMOPHAGOCYTIC SYNDROME AND TERMINAL LEUKEMIC TRANSFORMATION

We report a case of subcutaneous T-cell lymphoma that presented as rec urrent subcutaneous nodules, pancytopenia, and fever. Histopathologic examination revealed a dense infiltrate of markedly atypical lymphoid cells localized to the panniculus. These cells were identified as T ce lls by immunohistochemistry. There was associated karyorrhexis and fat necrosis. Hemophagocytosis was present both in the panniculus and in the bone marrow, with no tumor evident outside the subcutaneous tissue . Despite chemotherapy in conjunction with an autologous bone marrow t ransplant, the patient died after metastases, including explosive leuk emic transformation, developed. Review of the literature shows subcuta neous T-cell lymphoma to be a rare peripheral T-cell lymphoma, often m istaken initially as a benign panniculitis, that manifests an aggressi ve, fulminant presentation in approximately one half of the patients; the remainder transform into a high-grade malignancy after months to y ears. The hemophagocytic syndrome, thought to be a reactive T-cell pro cess mediated by cytokines, is a frequent complication of this lymphom a and is responsible for its poor prognosis. Our patient uniquely demo nstrated fatal leukemic transformation.