A Case of Subcutaneous Panniculitis-like
T Cell Lymphoma in Childhood |
Yoon Seok Choi1, Kyung Mi Shin1, Sung Chul Won1, Chuhl Joo Lyu1, Chang Hyun Yang1, Byung Soo Kim1, Moon Kyu Kim2 |
1Department of Pediatrics, College of Medicine, Yonsei University, Seoul, Korea 2Department of Pediatrics, College of Medicine, Aju University, Suwon, Korea |
소아에서 발생한 Subcutaneous Panniculitis-likeT Cell Lymphoma 1례 |
최윤석1, 신경미1, 원성철1, 유철주1, 양창현1, 김병수1, 김문규2 |
1연세대학교 의과대학 소아과학교실 2아주대학교 의과대학 소아과학교실 |
Correspondence:
Chuhl Joo Lyu, Email: cj@yumc.yonsei.ac.kr |
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Abstract |
Subcutaneous pannicultis-like T cell lymphoma is a rare cutaneous T cell lymphoma. It presents with multiple subcutaneous nodules or plaques involving the extremities 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 undefined 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 are suggestive of hemophagocytosis. Much of the mortality of this disease is due not to disseminated lymphoma with organ failure, but rather to 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. |
Key Words:
Subcutaneous panniculitis-like T cell lymphoma |
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