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

Kevin E. Salhany, William R. Macon, John K. Choi, Rosalie Elenitsas, Stuart R. Lessin, Raymond E. Felgar, Darren M. Wilson, Grzegorz K. Przybylski, John Lister, Mariusz A. Wasik, Steven H. Swerdlow

Research output: Contribution to journalArticle

280 Citations (Scopus)

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 Iobular panniculitislike 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 (CD3ε, CD45RO, or CD43) and T-cell receptors (TCR); nine expressed αβ TCRs and two expressed γδ TCRs. T- cell receptor-γ, TCRβ, or TCRδ genes were clonally rearranged in 8 of 10 cases studied. Both γδ SPTCLs expressed V(δ)2+ TCRs and were CD4-, CD8- and CD56+. CD56 was negative in seven of nine αβ SPTCLs and inconclusive in the other two. Six of nine αβ 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 αβ T-cells or γδ T-cells. The γδ SPTCLs appear to be preferentially derived from the V(δ)2+ subset. Subcutaneous panniculitis-like T-cell lymphoma may be rapidly fatal or indolent; local therapy may be appropriate for some patients.

Original languageEnglish (US)
Pages (from-to)881-893
Number of pages13
JournalAmerican Journal of Surgical Pathology
Volume22
Issue number7
DOIs
StatePublished - 1998
Externally publishedYes

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T-Cell Antigen Receptor
T-Lymphocytes
CD43 Antigens
CD3 Antigens
CD45 Antigens
Fat Necrosis
T-Cell Receptor Genes
Perforin
Histiocytes
T-Cell Lymphoma
Human Herpesvirus 4
In Situ Hybridization
Lymphoma
Necrosis
Radiotherapy
Lymphocytes
Phenotype
Drug Therapy
Skin
Subcutaneous panniculitis-like T-cell lymphoma

Keywords

  • CD56
  • Cutaneous T-cell lymphoma-γδ T-cell lymphoma
  • Cytotoxic T lymphocyte
  • Epstein-Barr virus
  • Perforin
  • Subcutaneous panniculitis-like T-cell lymphoma
  • TIA-1
  • V(δ)2 subset

ASJC Scopus subject areas

  • Anatomy
  • Pathology and Forensic Medicine

Cite this

Subcutaneous panniculitis-like T-cell lymphoma. Clinicopathologic, immunophenotypic, and genotypic analysis of alpha/beta and gamma/delta subtypes. / Salhany, Kevin E.; Macon, William R.; Choi, John K.; Elenitsas, Rosalie; Lessin, Stuart R.; Felgar, Raymond E.; Wilson, Darren M.; Przybylski, Grzegorz K.; Lister, John; Wasik, Mariusz A.; Swerdlow, Steven H.

In: American Journal of Surgical Pathology, Vol. 22, No. 7, 1998, p. 881-893.

Research output: Contribution to journalArticle

Salhany, KE, Macon, WR, Choi, JK, Elenitsas, R, Lessin, SR, Felgar, RE, Wilson, DM, Przybylski, GK, Lister, J, Wasik, MA & Swerdlow, SH 1998, 'Subcutaneous panniculitis-like T-cell lymphoma. Clinicopathologic, immunophenotypic, and genotypic analysis of alpha/beta and gamma/delta subtypes', American Journal of Surgical Pathology, vol. 22, no. 7, pp. 881-893. https://doi.org/10.1097/00000478-199807000-00010
Salhany, Kevin E. ; Macon, William R. ; Choi, John K. ; Elenitsas, Rosalie ; Lessin, Stuart R. ; Felgar, Raymond E. ; Wilson, Darren M. ; Przybylski, Grzegorz K. ; Lister, John ; Wasik, Mariusz A. ; Swerdlow, Steven H. / Subcutaneous panniculitis-like T-cell lymphoma. Clinicopathologic, immunophenotypic, and genotypic analysis of alpha/beta and gamma/delta subtypes. In: American Journal of Surgical Pathology. 1998 ; Vol. 22, No. 7. pp. 881-893.
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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 Iobular panniculitislike 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 (CD3ε, CD45RO, or CD43) and T-cell receptors (TCR); nine expressed αβ TCRs and two expressed γδ TCRs. T- cell receptor-γ, TCRβ, or TCRδ genes were clonally rearranged in 8 of 10 cases studied. Both γδ SPTCLs expressed V(δ)2+ TCRs and were CD4-, CD8- and CD56+. CD56 was negative in seven of nine αβ SPTCLs and inconclusive in the other two. Six of nine αβ 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 αβ T-cells or γδ T-cells. The γδ SPTCLs appear to be preferentially derived from the V(δ)2+ subset. Subcutaneous panniculitis-like T-cell lymphoma may be rapidly fatal or indolent; local therapy may be appropriate for some patients.",
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AU - Salhany, Kevin E.

AU - Macon, William R.

AU - Choi, John K.

AU - Elenitsas, Rosalie

AU - Lessin, Stuart R.

AU - Felgar, Raymond E.

AU - Wilson, Darren M.

AU - Przybylski, Grzegorz K.

AU - Lister, John

AU - Wasik, Mariusz A.

AU - Swerdlow, Steven H.

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N2 - 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 Iobular panniculitislike 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 (CD3ε, CD45RO, or CD43) and T-cell receptors (TCR); nine expressed αβ TCRs and two expressed γδ TCRs. T- cell receptor-γ, TCRβ, or TCRδ genes were clonally rearranged in 8 of 10 cases studied. Both γδ SPTCLs expressed V(δ)2+ TCRs and were CD4-, CD8- and CD56+. CD56 was negative in seven of nine αβ SPTCLs and inconclusive in the other two. Six of nine αβ 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 αβ T-cells or γδ T-cells. The γδ SPTCLs appear to be preferentially derived from the V(δ)2+ subset. Subcutaneous panniculitis-like T-cell lymphoma may be rapidly fatal or indolent; local therapy may be appropriate for some patients.

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KW - Epstein-Barr virus

KW - Perforin

KW - Subcutaneous panniculitis-like T-cell lymphoma

KW - TIA-1

KW - V(δ)2 subset

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