Table 1. Clinical characteristics of blastic plasmacytoid dendritic cell neoplasms patients classified according to their maturation-associated immunophenotypic profile.
| Blast cell phenotype | |||||
|---|---|---|---|---|---|
| Demographics | Total cases | Group 1 (immature) | Group 2 (intermediate) | Group 3 (mature) | P-value |
| n = 46 | n = 8 | n = 24 | n = 14 | ||
| Age (years)* | 61 ± 22 (8–91) | 68 ± 22 (25–91) | 56 ± 23 (8–85) | 68 ± 19 (11–83) | .05c |
| Children/Adults | 4/42 (9%/91%) | 0/8 (0%/100%) | 3/21 (13%/87%) | 1/13 (7%/93%) | NS |
| Sex (male/female) | 35/11 (76%/24%) | 6/2 (75%/25%) | 15/9 (63%/37%) | 14/0 (100%/0%) | .008c |
| Reason for consulting | |||||
| Routine blood analysis | 5/44 (11%) | 0/6 (0%) | 3/24 (13%) | 2/14 (14%) | NS |
| B & other general symptoms | 32/45 (71%) | 6/7 (86%) | 17/24 (71%) | 9/14 (64%) | NS |
| Bleeding | 6/45 (13%) | 1/7 (14%) | 3/24 (13%) | 2/14 (14%) | NS |
| Bone pain | 4/45 (9%) | 0/7 (0%) | 4/24 (17%) | 0/14 (0%) | NS |
| Organ involvement | 27/45 (60%) | 0/7 (0%) | 14/24 (58%) | 13/14 (93%) | ≤ .03a, b, c |
| Physical examination | |||||
| Adenopathies | 23/45 (51%) | 0/7 (0%) | 14/24 (58%) | 9/14 (64%) | .007a, b |
| Splenomegaly | 17/45 (38%) | 0/7 (0%) | 13/24 (54%) | 4/14 (29%) | .01a |
| Hepatomegaly | 10/45 (22%) | 0/7 (0%) | 7/24 (29%) | 3/14 (21%) | NS |
| Extramedullary involvement | 33/45 (73%) | 1/7 (14%) | 19/24 (79%) | 13/14 (93%) | ≤ .004a, b |
| - Skin1 | 29/45 (64%) | 1/7 (14%) | 16/24 (67%) | 12/14 (86%) | ≤ .02a, b |
| - CNS | 4/45 (9%) | 0/7 (0%) | 3/24 (13%) | 1/14 (7%) | NS |
| - Testis2 | 2/34 (6%) | 0/5 (0%) | 1/15 (7%) | 1/14 (7%) | NS |
| Clinical history | |||||
| Associated neoplasia | 3/44 (7%) | 0/6 (0%) | 1/24 (4%) | 2/14 (14%) | NS |
| Myelodysplastic syndrome | 5/32 (16%) | 2/6 (33%) | 1/19 (5%) | 2/7 (29%) | NS |
Results expressed as number of cases from all cases with available data and (percentage) or as *mean ± one standard deviation (range). Statistically significantly differences were found between agroup 1 group 2, bgroup 1 group 3 and cgroup 2 group 3. 1None showed mucosal involvement, 2only in male patients, both children. NS: no statistically significant differences (p > 0.05), CNS: central nervous system.