Table 1 Presenting clinical and laboratory characteristics and treatment details of 58 adult patients with blastic plasmacytoid dendritic cell neoplasm.
Variables | All patients n = 58 |
---|---|
Age in years, median (range), | 69 (58–76) |
 Age >70 years, n (%) | 25 (43) |
Male, n (%) | 46 (79) |
Hemoglobin, g/dl, median (range) | 11.6 (9.5–13.5) |
Leukocyte count × 109/L, median (range) | 4.4 (2.1–6.9) |
Platelet count × 109/L, median (range) | 132 (67–204) |
Bone marrow blast percentage, median (range) | 50 (1–95%) |
Site of involvement, n (%) | |
 Cutaneous | 37 (64) |
 Bone marrow | 37 (64) |
 Lymph node | 27 (47) |
 Central nervous system | 11(19) |
Abnormal karyotype, n (%) | 8/36 (22) |
ELN 2017 risk | |
Intermediate | 4/36 (11) |
Adverse | 4/36 (11) |
Mutations on NGS, n (%) | n = 9 |
 ASXL1 | 6 (67) |
 RUNX1 | 6 (67) |
 TET2 | 5 (56) |
 SRSF2 | 3 (33) |
 NRAS | 2 (22) |
 ZRSR2 | 1(11) |
First-line treatment, n (%) | 41 (71) |
 Hyper CVAD | 12 (21) |
 AML induction regimena | 8(14) |
 Lymphoma regimenb | 7 (12) |
 Tagraxofusp | 5(9) |
 Hypomethylating agent + venetoclax | 4(7) |
 Hypomethylating agent | 3(5) |
 Other chemotherapyc | 2 (3) |
Initial treatment response, n (%) | |
Complete remission (CR) with or without count recovery (CRi) | 24/41 (59) |
Partial remission (PR) | 2/41 (5) |
Relapse after first remission | 19/24 (79) |
Second-line treatment, n (%) | 19(33) |
 AML induction regimena | 7(12) |
 Tagraxofusp | 3 (5) |
 Lymphoma regimenb | 3(5) |
 Hypomethylating agent + venetoclax | 2 (5) |
 Hyper CVAD | 2(3) |
 Hypomethylating agent | 1 (2) |
 Other chemotherapyc | 1(2) |
Autologous stem cell transplant, n (%) | 3(5) |
Allogeneic stem cell transplant, n (%) | 15(26) |