Table 1 Presenting clinical and laboratory characteristics and treatment details of 58 adult patients with blastic plasmacytoid dendritic cell neoplasm.

From: Abnormal karyotype is an independent predictor of inferior survival in Blastic Plasmacytoid Dendritic Cell Neoplasm (BPDCN)

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)

  1. ELN European LeukemiaNet, NGS next generation sequencing, AML acute myeloid leukemia, Hyper CVAD hyper fractioned cyclophosphamide, vincristine, doxorubicin, dexamethasone alternating with high dose methotrexate and cytarabine.
  2. aAML induction regimen- 7 (cytarabine)+3 (daunorubicin/idarubicin), CLAM (cladribine, cytarabine, mitoxantrone), FLAM (fludarabine. cytarabine, mitoxantrone), mitoxantrone and cytarabine, ADE (cytarabine, daunorubicin, etoposide), high dose cytarabine monotherapy.
  3. bLymphoma regimen- CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone), ICE (cyclophosphamide, cisplatin, etoposide), cyclophosphamide, etoposide, and bleomycin.
  4. cOther chemotherapy- single agent fludarabine, bortezomib, or lenalidomide.