Among extranodal non-Hodgkin's lymphomas, primary cutaneous lymphomas
(CLs) represent a consistent group of B- and T-cell malignancies. We i
nvestigated the arrangement of Ig and T-cell receptor (TCR) genes, tog
ether with the involvement of several oncogenes and the tumor-suppress
or gene p53, in a panel of primary cutaneous B- and T-cell lymphomas (
CBCLs and CTCLs). Southern blot analysis was performed to detect rearr
angements of the Ig, c-myc, bcl-1, bcl-2, bcl-3, bcl-6, and the NFKB2/
lyt-10 genes in 52 cases of CBCLs and of the TCR, bcl-3, and NFKB2/lyt
-10 genes in 38 cases of CTCLs. tal-1 gene deletions were analyzed in
CTCLs by means of polymerase chain reaction (PCR). p53 gene mutations
were assayed using PCR, single-strand conformation polymorphism analys
is, and direct DNA sequencing in CBCL and CTCL cases. Clonal rearrange
ments of Ig genes or oncogenes were found in 25 of the 52 CBCLs. In pa
rticular, we detected rearrangements of the bcl-1 locus (2 cases), the
bcl-2 gene (2 cases), the NFKB2/lyt-10 gene (2 cases), and the bcl-6
gene (1 case); interestingly, 4 of these cases showed a germline arran
gement of the Ig genes. Clonal rearrangements of TCR genes were detect
ed in 37 of the 38 CTCLs. Rearrangements of the NFKB2/lyt-10 gene were
present in 2 cases and tal-1 gene deletions in 3 CTCL cases; 953 gene
mutations were detected in 1 CTCL case. Overall, our data indicate th
at(1) clonal rearrangement of Ig genes is frequently undetectable by m
eans of Southern blot in CBCLs ( 60%); (2) genetic lesions are involve
d in a limited but significant fraction of primary CLs showing a molec
ular marker of clonality (13/62; 20%); and (3) rearrangements of the b
cl-1, bcl-2, or bcl-6 loci, associated with specific subsets of nodal
lymphoid neoplasias, are rarely observed in CBCLs. Moreover, our resul
ts suggest that tal-1 gene deletions may play a pathogenetic role in n
on-acute T-cell malignancies and that, in the context of lymphoid mali
gnancies, CLs may represent a favorable target for the possible oncoge
nic potential of the NFKB2/lyt-10 gene. (C) 1995 by The American Socie
ty of Hematology.