Subcutaneous panniculitis-like T-cell lymphoma: clinicopathologic, immunophenotypic, and genotypic analysis of alpha/beta and gamma/delta subtypes.

Abstract

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon cutaneous lymphoma that has been proposed as a distinct clinicopathologic entity, but studies of SPTCL are limited. We studied the clinicopathologic, immunophenotypic, and genetic features of 11 SPTCLs. All cases had a variable admixture of pleomorphic small, medium, or large lymphocytes and histiocytes infiltrating the subcutis in a lobular panniculitis-like pattern. A granulomatous reaction was seen in three cases and erythrophagocytosis in four. Karyorrhexis and fat necrosis were present in all cases. Angioinvasion was seen in seven SPTCLs; four had areas of coagulation necrosis. All cases expressed T-cell-associated antigens (CD3epsilon, CD45RO, or CD43) and T-cell receptors (TCR); nine expressed alphabeta TCRs and two expressed gammadelta TCRs. T-cell receptor-gamma, TCRbeta, or TCRdelta genes were clonally rearranged in 8 of 10 cases studied. Both gammadelta SPTCLs expressed Vdelta2+ TCRs and were CD4-, CD8- and CD56+. CD56 was negative in seven of nine alphabeta SPTCLs and inconclusive in the other two. Six of nine alphabeta SPTCLs were CD8+; the CD4/CD8 phenotypes were indeterminate in the other three. Cytolytic granule-associated proteins were expressed by all SPTCLs (11 of 11 were TIA-1+, 4 of 4 were perforin+). In situ hybridization for Epstein-Barr virus-encoded RNA (EBER-1) was negative in all cases. Most patients responded to systemic chemotherapy or local radiation therapy. Seven patients are alive: four without disease (19-73 months) and three with disease (32-72 months); four died: three of disease (3-25 months) and one without disease (42 months). We conclude that SPTCLs are clonal, EBV-, cytotoxic T-cell lymphomas derived from alphabeta T-cells or gammadelta T-cells. The gammadelta SPTCLs appear to be preferentially derived from the Vdelta2+ subset. Subcutaneous panniculitis-like T-cell lymphoma may be rapidly fatal or indolent; local therapy may be appropriate for some patients.

• Authors: K E Salhany, W R Macon, J K Choi, R Elenitsas, S R Lessin, R E Felgar, D M Wilson, G K Przybylski, J Lister, M A Wasik, S H Swerdlow
• Journal: The American journal of surgical pathology (Am J Surg Pathol) Vol. 22 Issue 7 Pg. 881-93 (Jul 1998) ISSN: 0147-5185 [Print] United States
• PMID: 9669350 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Antigens, CD
• DNA, Neoplasm
• Epstein-Barr virus encoded RNA 1
• RNA, Viral
• Receptors, Antigen, T-Cell, alpha-beta
• Receptors, Antigen, T-Cell, gamma-delta

Topics

• Adolescent
• Adult
• Aged
• Aged, 80 and over
• Antigens, CD (analysis)
• CD4-Positive T-Lymphocytes (immunology)
• CD8-Positive T-Lymphocytes (immunology)
• DNA, Neoplasm (analysis)
• Female
• Gene Rearrangement, T-Lymphocyte (genetics)
• Genotype
• Herpesvirus 4, Human (genetics)
• Humans
• Immunoenzyme Techniques
• Immunophenotyping
• In Situ Hybridization
• Lymphoma, T-Cell, Cutaneous (chemistry, genetics, immunology, pathology)
• Male
• Middle Aged
• Panniculitis (genetics, immunology, pathology)
• RNA, Viral (analysis)
• Receptors, Antigen, T-Cell, alpha-beta (analysis, genetics)
• Receptors, Antigen, T-Cell, gamma-delta (analysis, genetics)
• Skin Neoplasms (chemistry, genetics, immunology, pathology)