Clinical, histologic, immunophenotypic, and genetic differences between PCFCCL with a diffuse large cell histology and PCLBCL-leg
. | PCFCCL, diffuse large cell . | PCLBCL-leg . | References . |
---|---|---|---|
Clinical features | |||
Median age at diagnosis (range) | 62 y (14-88 y) | 75 y (27-92 y) | Grange et al,7 Vermeer et al,8 Geelen et al,9 Goodlad et al11 |
Male-female ratio | 1:1 | 3:1 | Grange et al,7 Vermeer et al,8 Goodlad et al11 |
Site | Trunk; head (scalp) | Leg(s) | Willemze et al1 |
Treatment of first choice | Radiotherapy | Systemic chemotherapy | Willemze et al,1 Grange et al7 |
Dissemination | 11% | 50% | Grange et al,7 Vermeer et al8 |
5-year survival rate | > 95% | 52% | Grange et al,7 Vermeer et al8 |
Histopathology | |||
Cytomorphology | Large cleaved (85%) | Round (90%) | Willemze et al,3 Berti et al,4 Santucci et al,5 Grange et al,7 Vermeer et al8 |
Blastic transformation | Often* | No (de novo) | Willemze et al,3 Berti et al,4 Santucci et al,5 Grange et al,7 Vermeer et al8 |
Bcl-2 expression | -/+† | + | Geelen et al,9 Hoefnagel et al,10 Goodlad et al11 |
Bcl-6 expression | + | +/- | Hoefnagel et al,10 Goodlad et al,11 Paulli et al12 |
CD10 expression | - | - | Hoefnagel et al,10 Paulli et al12 |
Mum1/IRF4 expression | - | + | Present study, Paulli et al12 |
Genetics | |||
t(14; 18) | Absent | Absent | Geelen et al,9 Child et al,15 Cerroni et al,16 Hallermann et al18 |
CGH analysis | |||
loss 6q | 0 of 9 | 4 of 13 | Hallermann et al18 |
gain 18q | 0 of 9 | 8 of 13 | Hallermann et al18 |
FISH analysis | |||
IGH breakpoint | 0 of 6 | 7 of 14 | Sánchez-Beato et al29 |
CMYC breakpoint | 0 of 6 | 6 of 14 | Sánchez-Beato et al29 |
BCL6 breakpoint | 0 of 6 | 5 of 14 | Sánchez-Beato et al29 |
Gene expression profile | Germinal center B-cell type (GCB) | Activated B-cell type (ABC) | Present study |
. | PCFCCL, diffuse large cell . | PCLBCL-leg . | References . |
---|---|---|---|
Clinical features | |||
Median age at diagnosis (range) | 62 y (14-88 y) | 75 y (27-92 y) | Grange et al,7 Vermeer et al,8 Geelen et al,9 Goodlad et al11 |
Male-female ratio | 1:1 | 3:1 | Grange et al,7 Vermeer et al,8 Goodlad et al11 |
Site | Trunk; head (scalp) | Leg(s) | Willemze et al1 |
Treatment of first choice | Radiotherapy | Systemic chemotherapy | Willemze et al,1 Grange et al7 |
Dissemination | 11% | 50% | Grange et al,7 Vermeer et al8 |
5-year survival rate | > 95% | 52% | Grange et al,7 Vermeer et al8 |
Histopathology | |||
Cytomorphology | Large cleaved (85%) | Round (90%) | Willemze et al,3 Berti et al,4 Santucci et al,5 Grange et al,7 Vermeer et al8 |
Blastic transformation | Often* | No (de novo) | Willemze et al,3 Berti et al,4 Santucci et al,5 Grange et al,7 Vermeer et al8 |
Bcl-2 expression | -/+† | + | Geelen et al,9 Hoefnagel et al,10 Goodlad et al11 |
Bcl-6 expression | + | +/- | Hoefnagel et al,10 Goodlad et al,11 Paulli et al12 |
CD10 expression | - | - | Hoefnagel et al,10 Paulli et al12 |
Mum1/IRF4 expression | - | + | Present study, Paulli et al12 |
Genetics | |||
t(14; 18) | Absent | Absent | Geelen et al,9 Child et al,15 Cerroni et al,16 Hallermann et al18 |
CGH analysis | |||
loss 6q | 0 of 9 | 4 of 13 | Hallermann et al18 |
gain 18q | 0 of 9 | 8 of 13 | Hallermann et al18 |
FISH analysis | |||
IGH breakpoint | 0 of 6 | 7 of 14 | Sánchez-Beato et al29 |
CMYC breakpoint | 0 of 6 | 6 of 14 | Sánchez-Beato et al29 |
BCL6 breakpoint | 0 of 6 | 5 of 14 | Sánchez-Beato et al29 |
Gene expression profile | Germinal center B-cell type (GCB) | Activated B-cell type (ABC) | Present study |