Peripheral T-Cell Lymphoma: Aggressive Disease with Heterogeneous Immunotypes

Abstract

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.

HLA-DR (Ia) immune phenotype predicts outcome for patients with diffuse large cell lymphoma.

“…Most prior studies have explored the importance of determining the B-cell versus T-cell groups or the presence versus absence of surface immunoglobulins (3)(4)(5)(6)(7)(8). More recent trials have explored additional surface and nuclear antigens using newer monoclonal antibodies with some success including the activation factors, transferrin receptor (17), and HLA-DR (15,16), and proliferation antigens such as (18,19).…”

“…In an effort to more accurately predict outcome prior to treatment for patients with DLCL, investigators have explored the potential prognostic value of determining the immune phenotype of these NHLs. The results of those efforts are inconclusive with some studies showing no prognostic value (3)(4)(5) and others suggesting utility for determining the immune phenotype of DLCL (6)(7)(8). These disparate results may be related to any of several problems of past study designs including; small numbers of patients studied, comparisons between heterogenous histologic subgroups, and selection of monoclonal antibodies having little biological significance.…”

Peripheral T-Cell Lymphoma of the Scrotum

“…In regard to the latter, lymphoblastic lymphoma and mycosis fungoides are well-described entities. Recently, several investigators have reported the clinicopathologic features of peripheral T-cell lymphomas [2][3][4][5][6][7]. In general, patients are usually in their fifth to seventh decade of life, have B symptoms, and advanced stages (III of IV) of dis ease [2][3][4][5][6][7].…”

“…Recently, several investigators have reported the clinicopathologic features of peripheral T-cell lymphomas [2][3][4][5][6][7]. In general, patients are usually in their fifth to seventh decade of life, have B symptoms, and advanced stages (III of IV) of dis ease [2][3][4][5][6][7]. Histologically, such lymphomas are mainly in termediate-or high-grade, according to the Working For mulation Classification [8], Moreover, these lymphomas have a propensity for extranodal involvement, primarily of the skin, bone marrow, and lungs [2][3][4][5][6][7].…”

“…In general, patients are usually in their fifth to seventh decade of life, have B symptoms, and advanced stages (III of IV) of dis ease [2][3][4][5][6][7]. Histologically, such lymphomas are mainly in termediate-or high-grade, according to the Working For mulation Classification [8], Moreover, these lymphomas have a propensity for extranodal involvement, primarily of the skin, bone marrow, and lungs [2][3][4][5][6][7]. The present pa tient relapsed in the skin, whereas the case reported by Ray et al [1] had lung involvement.…”