Clinical and biological characteristics of the 2 MCL subtypes
| . | Conventional MCL . | Leukemic nonnodal MCL . |
|---|---|---|
| Male/Female113-116,118 | 3-4 | 1 |
| Clinical presentation113-116,118 | Lymphadenopathy | Leukemic* |
| Extranodal | Splenomegaly* | |
| Cell of origin109,110 | Naive-like | Memory-like |
| Morphology113-115,118,170 | Classic*/blastoid | Small cell*/blastoid/plasma cell differentiation (37%) |
| Phenotype113-115,118,171 | ||
| SOX11 | + | − |
| CD5 | + (90%-100%) | − (25%-50%) |
| CD200 | − (90%) | + (40%-90%) |
| Chromosomal instability109,113-116,118 | High | Low |
| Somatic mutations, %116 | ||
| ATM | 55 | 0 |
| TP53 | 25 | 25 |
| CDKN2A del | 20 | 0 |
| CCND1 | 18 | 86 |
| KMTD2/MLL2 | 18 | 0 |
| NSD2/WHSC1 | 15 | 0 |
| UBR5 | 14 | 0 |
| BIRC3 | 7 | 7 |
| NOTCH2 | 6 | 4 |
| NOTCH1 | 4 | 0 |
| TLR2 | 0 | 3 |
| Clinical behavior113-115,118 | Aggressive | Stable/indolent |
| . | Conventional MCL . | Leukemic nonnodal MCL . |
|---|---|---|
| Male/Female113-116,118 | 3-4 | 1 |
| Clinical presentation113-116,118 | Lymphadenopathy | Leukemic* |
| Extranodal | Splenomegaly* | |
| Cell of origin109,110 | Naive-like | Memory-like |
| Morphology113-115,118,170 | Classic*/blastoid | Small cell*/blastoid/plasma cell differentiation (37%) |
| Phenotype113-115,118,171 | ||
| SOX11 | + | − |
| CD5 | + (90%-100%) | − (25%-50%) |
| CD200 | − (90%) | + (40%-90%) |
| Chromosomal instability109,113-116,118 | High | Low |
| Somatic mutations, %116 | ||
| ATM | 55 | 0 |
| TP53 | 25 | 25 |
| CDKN2A del | 20 | 0 |
| CCND1 | 18 | 86 |
| KMTD2/MLL2 | 18 | 0 |
| NSD2/WHSC1 | 15 | 0 |
| UBR5 | 14 | 0 |
| BIRC3 | 7 | 7 |
| NOTCH2 | 6 | 4 |
| NOTCH1 | 4 | 0 |
| TLR2 | 0 | 3 |
| Clinical behavior113-115,118 | Aggressive | Stable/indolent |
These parameters are more frequent in the subtype indicated but not exclusive.