| 1 |
0-5 |
CD10+, BCL2+, and BLC2 translocation+ in 90% of cases |
| 2 |
6-15 |
CD10+, BCL2+, and BLC2 translocation+ in 90% of cases |
| 3A |
>15 |
Presence of centrocytes, CD10+, BCL2+, and BLC2 translocation+ in 75% of cases |
| 3B |
>15 |
Absence of centrocytes (diffuse areas of centroblasts), CD10+, BCL2+, and BLC2 translocation+ in few cases |
| Mutational profile |
| Gene |
% |
Function / Effect |
|
KMT2D
|
80-90 |
Loss of function; histone modification |
|
IgHV, IgLV
|
75-90 |
Gain of function; BCR signaling and proliferation |
|
CREBBP
|
33-70 |
Loss of function; histone modification |
|
BCL2
|
0.5 |
Gain of function; anti-apoptosis |
|
TNFRSF14
|
20-50 |
Loss of function; immune evasion |
|
BCL6
|
47 |
Gain of function; tumor progression |
|
H1-2, H1-4
|
44 |
Loss of function; chromatin remodeling |
|
RRAGC
|
17 |
gain of function; mTOR survival |
|
EZH2
|
7-30 |
Gain of function; histone modification |
|
TNFAIP3
|
2-26 |
Loss of function; survival |
| Prognosis |
| m7-FLIPI |
EZH2, ARID1A, MEF2B, EP300, FOXO1, CREBBP and CARD11
|
| Genetically-targeted therapy |
| Tazemetostat |
EZH2 inhibitor |
| Duvelisib |
PI3K inhibitor |
| Copanlisib |
PI3K inhibitor |
| Ibrutinib |
CARD11 and FOXO1 mutation+ cases |
| Vorinostat |
CREBBP and EP300 mutation+ cases |
| Pidilizumab |
PD-L1 |
