Subcutaneous panniculitis-likeT-cell lymphoma
Journal Title: Dermatologia Kliniczna - Year 2007, Vol 9, Issue 4
Abstract
Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare neoplasm derivingfrom cytotoxic T-cells primarily involving subcutaneous adipose tissue. Clinically, this malignancy presents as erythematosus, subcutaneous nodules and plaques affecting predominantly the trunk and lower extremities, mimicking inflammatory panniculitis. Two distinct clinical presentations have been identified. The indolentvariant is characterized by a chronic, reccurrent course. The aggressive variant is more often associated with haemophagocytic syndrome and a fatal outcome. According to the WHO-EORTC classification for cutaneous lymphomas the latter variant represents at least partially a new clinicopathological entity - a y/5 T-cell lymphoma. Histologically, the lymphoma infiltrates areconfined to the subcutaneous fat tissue and consistof a/P+, CD3+, CD4-, CD8+/-, lymphocytes expressing cytotoxicT-cell markersTIA and Granzyme B. To date, no consensus on the therapy has been established. Steroidotherapy has been considered as a sufficient treatment in the smoldering phase of the disease. In aggressive variants various chemotherapy regimens alone or combined with immunosuppressive agents, biological agents and radiotherapy have been employed.The autologous stem cell transplantation should be used in the refractory or recurrent aggressive SPTL.
Authors and Affiliations
Joanna Maj, Alina Jankowska-Konsur
Keywords
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