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primary cutaneous small/medium CD4+ T-cell lymphoma (PCSM-TCL)
Pathology:
- results from CD4+ T-cell proliferation
- high proliferative activity, demonstrated by Ki-67 staining & low CD8+ tumor lymphocytic response characterizes aggressive subgroup that progresses to disseminated disease & death
Clinical manifestations:
- frequently presents as a solitary plaque or tumor of 1-3 cm
a) favorable clinical prognosis
Laboratory:
- complete blood count (CDC)
- intense eosinophilia is a marker of indolent disease
- tissue biopsy Differential diagnsosis
1) absence of progression of these lesions from patch to plaque to tumor characterizes PCSM-TCL & distinguishes it from mycosis fungoides
Management:
- solitary plaque or tumor of 1-3 cm has a favorable clinical prognosis
- excision, radiotherapy, & topical steroids for indolent solitary lymphomas
- chemotherapy is reserved for the aggressive variant
General
unspecified primary cutaneous peripheral T-cell lymphoma
References
- Garcia-Herrera A et al.
Primary cutaneous small/medium CD4+ T-cell lymphomas:
A heterogeneous group of tumors with different
clinicopathologic features and outcome.
J Clin Oncol 2008 Jul 10; 26:3364.
PMID: 18541895