Table 1 Comparison among all patients in this cohort and MDS/MPN-RS-T.

From: Chronic myeloid neoplasms harboring concomitant mutations in myeloproliferative neoplasm driver genes (JAK2/MPL/CALR) and SF3B1

 

MDS/MPN-RS-T (n = 18)

MDS (n = 10)

p value (MDS vs MDS/MPN-RS-T)

MPN (n = 42)

p value (MPN vs MDS/MPN-RS-T)

MDS/MPN (n = 6)

p value (MDS/MPN vs MDS/MPN-RS-T)

NGS performed at the time of initial diagnosis

14 (78%)

7 (70%)

 

15 (36%)

 

5 (83%)

 

±Time (months) between initial diagnosis and NGS, median (range)

43.6 (19.9–106.6)

59.2 (12.3–86.7)

 

81.5 (35.7–377.4)

 

72.7

 

Male:female

8:10

7:3

0.2543

26:16

0.2614

5:1

0.166

Age (years), median (range)

67 (42–84)

69.5 (55–80)

0.6456

69.5 (35–90)

0.8197

75.5 (56–80)

0.424

Splenomegaly

4/18

0/10

0.2652

29/42

0.0015

3/6

0.3068

Hb (g/dL), median (range)

10.1 (7.7–12.9)

8.9 (6.6–11.3)

0.0734

9.1 (6.8–15.9)

0.3028

9.0 (6.7–13.1)

0.3429

Transfusion dependent

3/18

4/10

0.2075

16/41

0.1321

2/5

0.2907

ANC (×109/L), median (range)

5.3 (2.7–20.8)

1.7 (0.1–5.8)

0.0002

6.0 (0.8–35.9)

0.2366

5.1 (1.7–14.8)

0.8332

Platelets (×109/L), median (range)

768 (246–1838)

311 (46–399)

<0.0001

268 (29–1294)

<0.0001

567 (76–1144)

0.1765

RS (%), median (range)

30 (15–50)

30 (0–78)

0.8924

1 (0–70)

0.0004

5 (0–15)

0.0002

≥15% RS

16/16

7/9

0.1200

12/32

<0.0001

1/5

0.0008

Erythroid dysplasia

12/16

9/10

0.6169

10/40

0.0008

3/6

0.3341

Granulocytic dysplasia

2/15

6/10

0.028

2/40

0.2975

5/6

0.0055

Megakaryocytes

  

0.0005

 

0.0359

 

0.2498

  MDS like

2/17

9/10

 

0/40

 

3/6

 

  MPN like

11/17

0/10

 

36/40

 

2/6

 

  Mixed

3/17

0/10

 

4/40

 

1/6

 

  Normal-appearing

1/17

1/10

 

0/40

 

0/6

 

Myelofibrosis, MF2-3

5/16

0/7

0.0946

26/39

0.0162

3/6

0.4155

Abnormal karyotype

2/16

4/9

0.0726

14/40

0.0922

3/6

0.0616

Complex karyotype

0

1

 

3

 

1

 

JAK2 vs CALR vs MPL

14/3/1

8/0/2

0.2319

36/3/3

0.5258

3/2/1

0.4154

MPN-driver mutation, median VAF(%)

26.2 (5.5–45.2)

7.5 (0.6–53)

0.1273

44.7 (5–98.6)

0.0006

31 (5–53.3)

0.4727

MPN-driver mutation VAF < 10%

3/18

6/10

0.0346

2/42

0.1537

1/6

1

SF3B1 mutation, median VAF (%)

35 (10.2–48.7)

25.1 (3–48)

0.2199

39.1 (3.2–47)

0.1805

38.8 (23–47)

0.2938

SF3B1 mutation VAF < 10%

0/18

2/10

0.1190

4/42

0.3061

0/6

1

Mutation dominance, MPN-driver/SF3B1/No

2/6/9

3/6/1

0.0769

22/7/13

0.0151

1/3/2

0.7106

Other mutations

11/18

8/10

0.3051

21/42

0.4292

5/6

0.3173

Treatment information available

17

10

 

42

 

6

 

  Cytoreduction (hydroxyurea)

8

1

0.0912

5

0.0058

1

0.3401

  JAK2 inhibitor

2

0

0.5157

23

0.0032

1

1

  HMA

1

5

0.0152

4

1

1

0.4625

  Lenalidomide

2

3

0.3261

2

0.5712

2

0.2705

  Observation/supportive

6

2

0.6655

11

0.5346

1

0.6214

  SCT

2

0

0.5157

7

1

0

1

AML progression

0

1

NA

0

NA

1

NA

Median survival (months)

Not reached

Not reached

0.280

Not reached

0.331

Not reached

>0.999

  1. p values compared to MDS/MPN-RS-T. ± only for cases in which NGS was performed after initial diagnosis. Treatment prior to NGS: MDS (2HMA, 1 lenalidomide, 1 observation); MPN (15 JAK2 inhibitors, 2 lenolidomide, 5 hydroxyurea, 4 HMA), MDS/MPN (1 lenolidomide); MDS/MPN-RS-T (3 hydroxyurea, 1 supportive).
  2. MDS/MPN-RS-T myelodysplastic/myeloproliferative neoplasm with ring sideroblasts and thrombocytosis, MDS myelodysplastic syndrome, MPN myeloproliferative neoplasm, Hb hemoglobin, ANC absolute neutrophil count, RS ring sideroblasts, megs megakaryocytes, MF myelofibrosis, VAF variant allelic frequency, AML acute myeloid leukemia.