Subcutaneous panniculitis-like T-cell lymphoma: presentation of 2 cases and observations.
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.
Papenfuss
Jason S
JS
Department of Internal Medicine, University of Utah Health Sciences Center, Salt Lake City, USA. jpaps1212@hotmail.com
Aoun
Patricia
P
Bierman
Philip J
PJ
Armitage
James O
JO
D017667:Adipocytes / Q000378:metabolism; D018414:CD8-Positive T-Lymphocytes / Q000378:metabolism; D003593:Cytoplasm / Q000378:metabolism; D018450:Disease Progression; D005260:Female; D053804:Granzymes; D006801:Humans; D007150:Immunohistochemistry; D016399:Lymphoma, T-Cell / Q000175:diagnosis* / Q000473:pathology; D016410:Lymphoma, T-Cell, Cutaneous / Q000175:diagnosis / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009336:Necrosis; D015434:Panniculitis / Q000175:diagnosis* / Q000473:pathology; D016879:Salvage Therapy; D012697:Serine Endopeptidases / Q000378:metabolism; D012867:Skin / Q000473:pathology
C509213:GZMB protein, human; D053804:Granzymes; D012697:Serine Endopeptidases