Peripheral T-cell lymphoma: aggressive disease with heterogeneous immunotypes.
Eleven patients with mature or peripheral T-cell lymphoma (PTL), other than mycosis fungoides, were identified using an extensive battery of T- and B-cell markers. Eight cases had a histologic diagnosis of either diffuse large cell or mixed lymphoma, three of small cell type. All cases had one or more "mature" T-antigens and an absence of B- and immature T-antigens. Assessment of T-antigens included E-rosettes (Er), anti-Leu 1-7 and Tdt. The authors delineated striking heterogeneity of T-antigen expression: 9 different immunotypes in 11 cases. Subset T-antigen assessment indicated T-helper neoplastic cells in five cases and T-suppressor in two. The remaining four had universal T-antigens alone. Seven cases appeared to have "novel" T-phenotypes not corresponding to normal T-ontogeny phenotypes. Novel or idiosyncratic phenotypes may be a key characteristic of PTL. Since no single T-antigen, including Er and Er receptor (Leu-5), was expressed in all cases, a battery of monoclonal antibodies is necessary to detect PTL. Clinically, the authors found PTL unexpectedly aggressive, despite mature immunotype. Most patients were elderly (median age 69); all had extranodal disease with cutaneous involvement (six cases) most frequent. Responses to chemotherapy frequently proved transient, with median survival of nine months. A fulminant course was noted even with localized presentation. Clinical outcome suggests PTL requires new therapeutic strategies.
Grogan
T M
TM
Fielder
K
K
Rangel
C
C
Jolley
C J
CJ
Wirt
D P
DP
Hicks
M J
MJ
Miller
T P
TP
Brooks
R
R
Greenberg
B
B
Jones
S
S
D000328:Adult; D000368:Aged; D000911:Antibodies, Monoclonal; D000954:Antigens, Surface / Q000032:analysis; D005260:Female; D006801:Humans; D008198:Lymph Nodes / Q000276:immunology / Q000473:pathology; D008223:Lymphoma / Q000276:immunology* / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000276:immunology / Q000473:pathology; D008228:Lymphoma, Non-Hodgkin / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D012397:Rosette Formation; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology; D013601:T-Lymphocytes / Q000145:classification / Q000276:immunology; D018127:Tumor Necrosis Factor Receptor Superfamily, Member 7
D000911:Antibodies, Monoclonal; D000954:Antigens, Surface; D018127:Tumor Necrosis Factor Receptor Superfamily, Member 7