Comparative study of cutaneous T-cell lymphoma and adult T-cell leukemia/lymphoma: Clinical, histopathologic, and immunohistochemical analyses

Abstract

An important disease entity distinct from cutaneous T-cell lymphoma (CTCL) in Japan is adult T-cell leukemia/lymphoma (ATL), which usually shows the same phenotype as CTCL, i.e., a helper/inducer T-cell phenotype (CD4+CD8-), and usually involves the skin. Clinically, both CTCL and ATL are heterogeneous in nature. In this study, we demonstrated differences between CTCL and ATL in terms of clinical and immunopathologic cell surface features. In patients with ATL, the predominant clinical findings were peripheral lymph node involvement, skin lesions, hepatosplenomegaly, leukemic manifestations, and an aggressive course. In patients with CTCL, by contrast, only skin lesions predominated at the onset of the disease and a relatively good prognosis was demonstrated. Phenotypic heterogeneity of ATL in the skin, i.e., CD4-CD8-, CD4+CD8-, and CD4-CD8+, was demonstrated. Expression of Leu8, CD7 (Leu9), and CD45RA (2H4) was high in both the skin-infiltrating ATL cells and peripheral blood and lymph node ATL cells compared with that in the skin-infiltrating CTCL cells. Expression of CD25 (IL-2R), CD71 (OKT9), HLA-DR, and HLA-DQ was higher in the skin-infiltrating ATL cells than in CTCL cells. Expression of CD29 (4B4) was high, and that of CD45RA (2H4) was low in both the skin-infiltrating ATL and CTCL cells compared with the peripheral blood and lymph node ATL cells. Expression of CD45RO (UCHL-1) was not significantly high in the skin-infiltrating CTCL cells compared with that in ATL cells. The most significant phenotypic difference between ATL cells and CTCL cells was the expression of Leu8 (lymph node homing receptor), CD7 and CD25 antigens on the cell surface, and the main phenotypic difference between skin-infiltrating ATL and CTCL cells and peripheral blood and lymph node ATL cells was the expression of CD29 and CD45RA. These findings confirm that the difference in antigen expression on the cell surface might reflect the clinical features of ATL and CTCL, and suggest that the predominant phenotype of peripheral blood and lymph node ATL cells is that of naive, relatively immature or activated T-cells, and that CTCL cells are previously activated (memory) T-cells. In other words, CTCL cells do not share the same origin as ATL cells. These observations support the concept that ATL is a disease distinct from CTCL.

Role of the Fas/Fas ligand pathway in apoptotic cell death induced by the human T cell lymphotropic virus type I Tax transactivator.

“…In the light of our findings, it is plausible that the Fas\FasL pathway also plays a prominent part in the pathogenesis of ATL. Fas antigen is preferentially expressed on the surface of CD4 + and CD45RO + cells which are a major target for in vivo HTLV-I infection, thereby providing a receptor pool which effectively may be activated by FasL induced as a result of virus infection (Debatin et al, 1990 ;Miyawaki et al, 1992 ;Nagatani et al, 1990 ;Richardson et al, 1990 ;Shirono et al, 1989 ;Worner et al, 1990). Elimination of infected cells through apoptosis triggered by Fas-FasL interaction could provide an explanation for longterm latency of HTLV-I infection and a characteristically low frequency of cells expressing virus in infected individuals.…”

Cutaneous T-cell lymphoma in skin of color

“…The diagnosis of ATL leads to a therapy algorithm distinct from MF, even though many of the treatments for the chronic and smoldering phases are also used in MF patients. 36 …”

Mycosis fungoides: A case report

“…A viral etiology has been suspected because of certain similarities to HTLV-1 associated adult T cell leukemia-lymphoma but has not been proved as yet [6]. The majority of the cases occur in adult males but adolescents can also be affected [7].…”