소아에서 발생한 Subcutaneous Panniculitis-like T Cell Lymphoma 1례

Abstract

Subcutaneous panniculitis-like T cell lymphoma is a rare cutaneous T cell lymphoma. It presents with multiple subcutaneous nodules or plaques involving the exremities or trunk, and with constitutional symptoms that include fever, malaise, fatigue, myalgia, chills and weight loss. Histologically, the lesions of this disease are reminiscent of panniculitis and are composed of a mixture of small and large atypical lymphoid cells infiltrating between adipocytes. The optimal treatment for this disease is underfined and prognosis of this disease is poor, even when treated with multiagent chemotherapy regimens considered optimal for agressive lymphoma of other types. Poor prognosis factors include clinical features such as anemia, leukocytopenia, hepatosplenomegaly, lymphadenopathy and coagulopathy, which re suggestive of hemophagocytosis. Much of the mortality of this disease is due not to disseminated lymphoma with organ failure, but ratherto complications of the cytopenias associated with the hemophagocytic syndrome. We report a case of subcutaneous panniculitis-like T cell lymphoma in a 12 year-old boy who presented with initial complaints of fever and multiple subcutaneous nodules, and briefly review the related literature.