CUTANEOUS TYPE OF ADULT T-CELL LEUKEMIA LYMPHOMA IN A FRENCH WEST-INDIAN WOMAN - CLONAL REARRANGEMENT OF T-CELL RECEPTOR BETA-GENE AND GAMMA-GENE AND MONOCLONAL INTEGRATION OF HTLV-I PROVIRAL DNA IN THE SKIN INFILTRATE

A 45-year-old woman, a native of the French West Indies who had lived in France since 1973, developed multiple cutaneous plaques and nodules in 1987. Histopathologic studies revealed dermal infiltration with mature activated T cells (CD4+, CD25+, DR+) with nuclear convolutions and epidermatotropism. High titers of specific human T lymphotropic virus (HTLV)-I antibodies were detected in the serum. Molecular analysis of DNA extracted from the skin tumor biopsy specimen showed a clonal integration of an HTLV-I provirus and a T-cell clonal population as demonstrated by T-cell receptor β and γ gene rearrangement studies. Neither HTLV-I provirus nor T-cell receptor rearrangements were detected in peripheral blood mononuclear cells DNA despite the presence of rare adult T cell leukemia cells (< 1 %) and a small excess of DR-expressing cells, and detection of HTLV-I Pol and Px sequences by in vitro gene amplification. In this case only gene analysis of the skin lesions made possible an early diagnosis of a cutaneous adult T cell leukemia. This illustrates the need for such molecular studies to differentiate, in HTLV-I seropositive patients from endemic areas, a HTLV-I—induced T cell lymphoma from HTLV-I—nonrelated cutaneous T cell lymphomas. © 1990, American Academy of Dermatology, Inc.. All rights reserved.