Subcutaneous Panniculitis-like T cell Lymphoma with Hemophagocytic Syndrome / 대한피부과학회지

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.

Advances in etiology and pathogenesis of hemophagocytic syndrome / 医学研究生学报

Hemophagocytic syndrome (HPS) is a clinicopathologic entity occurs as a consequence of uncontrolled dysregulated cellular immune reactivity caused by a number of different underlying diseases,characterized by hypercytokinemia and systemic proliferation of benign hemophagocytic cells. Hemophagocytic syndrome embraces both primary/inherited HPS and secondary/reactive HPS. Mutations in perforin gene have been identified in some inherited HPS patients,there may be other genetic abnormalities also responsible for the disease. The causes of secondary HPS are diverse,including infections,malignancy and other unknown underlying diseases. A great advance in pathogenesis of HPS has been made and the results of recent research suggest that onset of HPS is closely associated with deficiency in lymphocyte mediated cytotoxicity and lowing apoptosis of activated immune cells.