TY - JOUR
T1 - Subcutaneous panniculitis-like T-cell lymphoma
T2 - Presentation of 2 cases and observations
AU - Papenfuss, Jason S.
AU - Aoun, Patricia
AU - Bierman, Philip J.
AU - Armitage, James O.
PY - 2002/12
Y1 - 2002/12
N2 - Subcutaneous panniculitis-like T-cell lymphoma is a rare tumor of primary cutaneous origin representing far < 1% of all non-Hodgkin's lymphomas. The disease typically follows a distinctive, indolent course of recurrent, self-healing subcutaneous nodules. These nodules mimic lipomas clinically, while histologically resembling a panniculitis. Alternatively, a rapidly progressive course might be seen with subcutaneous nodules accompanied by constitutional symptoms and, in some cases, the development of a potentially fatal hemophagocytic syndrome with significant cytopenia. This tumor is widely regarded as a tumor of CD8+ cytotoxic T cells with the presence of cytotoxic proteins, T-cell-restricted intracellular antigen, and granzyme B commonly demonstrated. A number of modalities have been reported in the treatment of this tumor, with varying degrees of success. In this report, we present 2 cases of subcutaneous panniculitis-like T-cell lymphoma with variable clinical courses. We also review the literature of this unusual lymphoma.
AB - Subcutaneous panniculitis-like T-cell lymphoma is a rare tumor of primary cutaneous origin representing far < 1% of all non-Hodgkin's lymphomas. The disease typically follows a distinctive, indolent course of recurrent, self-healing subcutaneous nodules. These nodules mimic lipomas clinically, while histologically resembling a panniculitis. Alternatively, a rapidly progressive course might be seen with subcutaneous nodules accompanied by constitutional symptoms and, in some cases, the development of a potentially fatal hemophagocytic syndrome with significant cytopenia. This tumor is widely regarded as a tumor of CD8+ cytotoxic T cells with the presence of cytotoxic proteins, T-cell-restricted intracellular antigen, and granzyme B commonly demonstrated. A number of modalities have been reported in the treatment of this tumor, with varying degrees of success. In this report, we present 2 cases of subcutaneous panniculitis-like T-cell lymphoma with variable clinical courses. We also review the literature of this unusual lymphoma.
KW - Alpha
KW - Beta
KW - Delta
KW - Gamma
KW - Granzyme B.
KW - Hemophagocytic syndromeCytotoxi T-cell
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U2 - 10.3816/CLM.2002.n.024
DO - 10.3816/CLM.2002.n.024
M3 - Article
C2 - 12521396
AN - SCOPUS:0036957116
VL - 3
SP - 175
EP - 180
JO - Clinical Lymphoma, Myeloma and Leukemia
JF - Clinical Lymphoma, Myeloma and Leukemia
SN - 2152-2669
IS - 3
ER -