Martine Bagot

Blastic NK-cell lymphomas (agranular CD4+CD56+ hematodermic neoplasms): a review.

Blastic natural killer (NK) cell lymphoma (also termed CD4+CD56+ hematodermic neoplasm) is a recently described entity, with the first case reported in 1994. It was suggested initially that the disease originates from NK cells. Since 1994, single cases and a few small series have been published. In this review, data from the literature and a series of 30 cases from the French and Dutch study groups on cutaneous lymphomas are discussed. The major clinical, histopathologic, and phenotypic aspects of the disease and diagnostic criteria and data suggesting a plasmacytoid dendritic cell origin for the tumor cells are provided.

Statistical evaluation of diagnostic and prognostic features of CD30+ cutaneous lymphoproliferative disorders : A clinicopathologic study of 65 cases

Several clinical and histopathologic features of 65 CD30+ cutaneous lymphoproliferations were evaluated for their diagnostic value between CD30+ primary versus secondary cutaneous lymphomas and for their prognostic significance. Primary cutaneous disease, spontaneous regression, and absence of extracutaneous spreading (but not age < or =60 years) were associated with a better prognosis. Epithelial membrane antigen, BNH9, CD15 or CBF.78 antigen were expressed in all types of cutaneous lymphoproliferations. However, epithelial membrane antigen immunoreactivity was more frequently expressed in CD30+ secondary cutaneous large-cell lymphoma. Among CD30+ primary cutaneous large-cell lymphoma, CD15 expression was only seen in localized skin lesions. P53 expression was not associated with spontaneous regression, extracutaneous spreading, or survival. Nested reverse transcriptase-polymerase chain reaction allowed the detection of NPM-ALK transcripts in 10 of 26 CD30+ primary and in 3 of 11 secondary cutaneous large-cell lymphomas. The ALK protein was detected in only 1 of 50 primary and in 4 of 15 secondary cutaneous CD30+ lymphoproliferations. In CD30+ primary cutaneous lymphoproliferation, NPM-ALK transcripts might be expressed by very rare normal or tumoral cells that are undetectable by immunohistochemistry. However, the expression of either NPM-ALK transcripts or ALK-protein was not correlated with prognosis or age in CD30+ cutaneous lymphoproliferations.

T Cell Receptor Variable Region Gene Expression in Cutaneous T-Cell Lymphoma

T lymphocytes interact with antigen and major histocompatibility complex via a specific T-cell receptor (TCR). Monoclonal antibodies to the variable region of TCR gene products have been produced that identify minor populations of normal peripheral blood T lymphocytes. These antibodies may detect clonal T-cell proliferations which express the same Vβ gene. An initial report using two anti-V region antibodies suggested that cutaneous T-cell lymphomas preferentially express the Vβ8 gene. However, several other studies with a larger panel of seven antibodies have not confirmed the restricted use of some Vβ segments. Indeed, we and others have found that, using this antibody panel, only a minority of cutaneous T-cell lymphomas was stained by one of these anti-V region antibodies, although, in two cases, it allowed us to demonstrate the strictly epidermotropic localization of clonal proliferation in plaque stage mycosis fungoides. Therefore, at the present time, immunohistological staining with these antibodies cannot replace molecular biological methods, either for the demonstration of clonality in T-cell lymphoma, or for distinguishing between benign lymphoid infiltrates and malignant lymphoma. Their usefulness as potentially virtually clonotypic markers in T-cell lymphoma needs to be reevaluated when a larger panel of antibodies becomes available.