For cases of SPTCL with a course expected to be aggressive (i.e., especially cases with expression of a TCR g/d+/CD56+ phenotype), multiagent chemotherapy, possibly followed by allogeneic BMT may be useful.

Figure 3 Subcutaneous panniculitis-like T-cell lymphoma: rimming of adipocytes by medium sized pleomorphic tumor cells and collections of nuclear fragments (bean bag cells).

Subcutaneous panniculitis-like T-Cell lymphoma

Clinical features

Location on lower extremities and trunk Deep-seated plaques or nodules which may ulcerate Histological features

Subcutaneous lymphocytic infiltrate. Minimal dermal or epidermal involvement. Rimming of fat lobules by pleomorphic medium to large lymphocytes. Karyorrhexis with macrophages, erythrophagocytosis Immunophenotype

CD2+, CD3+, CD5+, CD4-/+, CD8+/-, CD43+, TIA-1+, Granzyme B+ and perforin+

TCR a/b (bF1+)>TCR g/d (TCRd-1+) > CD4+ CD56+>CD30+ Molecular biology

Clonal TCR rearrangement in most cases Treatment

Not standardized. Clinical course and prognosis Indolent slowly progressive course more common in TCR a/b (bF1+) cases. Rapid and aggressive course with fatal outcome in TCR g/d (TCRd-1+) cases. Hemophagocytic syndrome with systemic signs in some patients

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