Subcutaneous panniculitis-like T cell lymphoma with hemophagocytic syndrome

Subcutaneous pnniculitis-like T cell lymphoma (SPTCL) is a rare subtype of cutaneous T cell lymphoma which clinically and histopathologically mimics benign panniculitis. It presents as tender erythematous, subcutaneous nodules or plaques on the extremities and trunk, and displays systemic symptoms such as weight loss, fever, chills, fatigue or myaligia. The association of hemophagocytic syndrome (HPS) portends a poor prognosis. We report a case of SPTCL that occurred in a 20-month-old girl. She presented with multiple erythematous subcutaneous nodules on the face, right thigh, buttocks and trunk, plus fever and vomiting. Histopathological examination of the subcutaneous nodules revealed a dense infiltration of atypical T-lymphoid cells in the subcutaneous fat layer, with rimming of fat cells, numerous karyorrhetic bodies and histiocytes, phagocytosed apoptotic cells and Bean bag cells In spite of combination chemotherapy, she died of disseminated intravascular coagulation due to HPS.