TRANSFORMATION OF MYCOSIS-FUNGOIDES - T-CELL RECEPTOR-BETA GENE ANALYSIS DEMONSTRATES A COMMON CLONAL ORIGIN FOR PLAQUE-TYPE MYCOSIS-FUNGOIDES AND CD30-CELL LYMPHOMA( LARGE)
Gs. Wood et al., TRANSFORMATION OF MYCOSIS-FUNGOIDES - T-CELL RECEPTOR-BETA GENE ANALYSIS DEMONSTRATES A COMMON CLONAL ORIGIN FOR PLAQUE-TYPE MYCOSIS-FUNGOIDES AND CD30-CELL LYMPHOMA( LARGE), Journal of investigative dermatology, 101(3), 1993, pp. 296-300
it is well recognized that patients with classical mycosis fungoides (
MF) may develop a large-cell lymphoma (LCL), a phenomenon known as ''t
ransformation.'' An unresolved issue regarding the transformation of M
F is whether MF and LCL represent two separate lymphomas or whether th
ey are derived from the same T-cell clone. We report the clinicopathol
ogic, immunophenotypic, and immunogenotypic analysis of MF and LCL in
a white male. He developed a sh at age 51 that was diagnosed at age 56
as clinical stage IA patch/plaque MF. After topical nitrogen mustard
and total skin electron beam therapy for progressive generalized CD3+C
D4+ patch/plaque-lesions, he developed nodules of Ki-1+ (CD30+) T-LCL
at age 72. Southern blot analysis of DNA digested with Bg/II or BamHI
and probed with a T-cell receptor (TCR)-beta gene Jbeta1/Jbeta2 probe
showed a single, identical rearranged band in both the MF and LCL skin
lesions that had been obtained 4 years apart. Vbeta gene family - spe
cific gene amplification assays demonstrated dominant Vbeta6 PCR produ
cts in both types of lesions. These PCR products and lesional cDNA exh
ibited a monoclonal pattern when amplified with consensus TCR-beta gen
e VDJ joint primers and electrophoresed under conditions that allowed
the resolution of Small differences in size. Furthermore, sequence ana
lysis of the Vbeta6 PCR products amplified from both the MF and LCL le
sions showed an identical nucleotide sequence involving Vbeta6.4, Dbet
a1.1, Jbeta1.2, and Cbeta1. These findings indicate that both the MF a
nd the LCL in this patient arose from the same T-cell clone and that t
hese diseases developed at a stage in the clone's differentiation subs
equent to rearrangement of the TCR-beta gene.