Table 1 Baseline clinicopathological characteristics of the study group.
Characteristics | BPDCN-C1 (n = 29) | BPDCN-C2 (n = 25) |
|---|---|---|
Age (yrs.; median (range)) | 70 (15–91) | 74 (42–90) |
Sex | ||
Female | 6 (21%) | 8 (32%) |
Male | 23 (79%) | 17 (68%) |
Manifestation | ||
Skin (histologically confirmed) | 20 (69%) | 14 (56%) |
Skin (suspected not biopsied) | 6 (21%) | 7 (28%) |
Bone marrow | 8 (28%) | 8 (32%) |
0 EN-sites | 3 (10%) | 3 (12%) |
1–2 EN sites | 26 (90%) | 19 (76%) |
>2 EN sites | – | 3 (12%) |
Stage (Ann Arbor) | ||
I/II | 2/16 (13%) | 3/16 (19%) |
III/IV | 14/16 (87%) | 13/16 (81%) |
ECOG PS | ||
0–1 | 8/10 (80%) | 3/11 (27%) |
≥2 | 2/10 (20%) | 8/11 (73%) |
B-symptoms | ||
No | 6/14 (43%) | 4/15 (27%) |
Yes | 8/14 (57%) | 11/15 (73%) |
Immunohistochemistry | ||
BPDCN-specific | 29/29 (100%) | 25/25 (100%) |
CD56+ | 29/29 (100%) | 25/25 (100%) |
CD123+ | 29/29 (100%) | 25/25 (100%) |
TCL1 | 27/28 (96%) | 17/23 (74%) |
Immature lineage marker | 21/29 (72%) | 19/24 (79%) |
CD34 | 1a/29 (3%) | 4a/24 (17%) |
TdT+ | 21/29 (72%) | 16/23 (70%) |
T-lineage markers | 28/29 (96%) | 24/25 (96%) |
CD2+ | 5/22 (23%) | 7/16 (44%) |
CD3+ | 4/26 (15%) | 5/23 (22%) |
CD4+ | 28/29 (96%) | 23/25 (92%) |
B-lineage marker CD79A+ | 23/28 (82%) | 20/23 (87%) |
Myeloid-lineage markers | 24/26 (92%) | 21/23 (91%) |
CD33+ | 24/26 (92%) | 20/23 (87%) |
CD117+ | 2/26 (8%) | 9/23 (39%) |
MPO+ | 1a/28 (4%) | 4a/23 (17%) |
Ki-67 (median, range) | 60% (30–90%) | 50% (25–90%) |
