Table 1.
Clinical and biological features of test and validation SMZL series
| Variables | Test cohort N (%) | Validation cohort N (%) |
|---|---|---|
| Material analyzed obtained from spleen | 90/98 (92%) | 36/36 (100%) |
| Material analyzed obtained from peripheral blood | 8/98 (8%) | 0/36 (0%) |
| Age, y (median, range) | 68 (30-91) | 66 (52-77) |
| Sex (male) | 49/92 (53%) | 7/23 (30%) |
| B symptoms | 14/45 (31%) | 4/22 (18%) |
| Bone marrow involvement | 50/52 (96%) | 31/31 (100%) |
| Peripheral blood involvement | 29/44 (66%) | 21/23 (91%) |
| Stage (III, IV) | 58/59 (98%) | 23/23 (100%) |
| IILSS (high + intermediate) | 22/36 (61%) | 8/19 (42%) |
| NOTCH2 mutation status | 7/37 (19%) | 6/33 (18%) |
| Notch pathway mutation status | 12/37 (32%) | 6/33 (18%) |
| NF-κB pathway mutation status | 8/31 (26%) | 4/21 (19%) |
| DNA-remodeling genes mutation status | 2/3 (67%) | 0/2 (0%) |
| TP53 mutation status | 4/31 (13%) | 2/21 (10%) |
| 7q31-32 loss | 14/66 (21%) | 11/36 (31%) |
| 17p loss | 10/66 (15%) | 6/36 (17%) |
| High-M phenotype | 21/98 (21%) | 12/36 (33%) |
| KM3 phenotype | 28/98 (29%) | 12/36 (33%) |
| IGHV1-02 usage | 12/65 (18%) | 4/22 (18%) |
| LDH increased | 15/40 (38%) | 4/22 (18%) |
| HCV status | 6/48 (13%) | 7/36 (19%) |
| Histologic transformation to high-grade lymphoma | 2/43 (5%) | 3/17 (18%) |
| Dead | 28/91 (31%) | 6/36 (17%) |
| Overall survival, mo (median, range) | 69.20 (3-223) | 58.10 (2-194) |
| Splenectomy | 34/50 (68%) | 3/24 (13%) |
| Treatment with CHT | 22/49 (45%) | 17/24 (44%) |
| GEP available | 10/98 (10%) | 0/36 (0%) |
CHT, chemotherapy; GEP, gene expression profiling; HCV, hepatitis C virus; IILSS, Intergruppo Italiano Linfomi Score for SMZL, including hemoglobin, LDH and albumin levels; LDH, lactate dehydrogenase.
The majority of patient samples were splenic biopsies (94%, 126/134) and 8 of 134 cases represented peripheral blood specimens (6%).