Cutaneous type of adult T cell leukemia/lymphoma in a French West Indian woman. Clonal rearrangement of T-cell receptor beta and gamma genes 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 epidermatotropisim. 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 beta and gamma 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 (less than 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.
Gessain
A
A
Laboratorie d'Hématologie Moléculaire, Hôpital Saint-Louis, Paris France.
Moulonguet
I
I
Flageul
B
B
Perrin
P
P
Capesius
C
C
D'Agay
M F
MF
Gisselbrecht
C
C
Sigaux
F
F
Civatte
J
J
D015703:Antigens, CD / Q000032:analysis; D015139:Blotting, Southern; D002478:Cells, Cultured; D004279:DNA, Viral / Q000235:genetics; D005260:Female; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor / Q000235:genetics; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000235:genetics; D015486:HTLV-I Antigens / Q000032:analysis; D015368:Human T-lymphotropic virus 1 / Q000235:genetics; D006801:Humans; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000276:immunology / Q000382:microbiology / Q000473:pathology*; D008875:Middle Aged; D010641:Phenotype; D011533:Proviruses / Q000235:genetics; D012867:Skin / Q000382:microbiology / Q000473:pathology; D012878:Skin Neoplasms / Q000276:immunology / Q000382:microbiology / Q000473:pathology*
D015703:Antigens, CD; D004279:DNA, Viral; D015486:HTLV-I Antigens