Table 1 Table showing phenotypic and genomic differences among patients with WHO-defined MDS/MPN-RS-T and MDS/MPN-U patients with BM RS% ≥ 15 (MDS/MPN-U-RS).

From: Myelodysplastic/myeloproliferative neoplasms with ring sideroblasts and thrombocytosis (MDS/MPN-RS-T): Mayo-Moffitt collaborative study of 158 patients

Variable; Median value (range or %)

MDS/MPN-RS-T (n = 158)

MDS/MPN-U-RS (n = 25)

P-value

Age (years)

71 (38–94)

71 (37–86)

0.9

Age ≥70 (years)

96 (61)

13 (52)

0.4

Males (%)

82 (52)

9 (36)

0.1

Clinical parameters

Evaluable = 78

Evaluable = 25

 

Hb; g/dL

9.5 (6.6–14.5)

9 (5.6–12.7)

0.2

Hb ≤10 g/dL

51 (65)

18 (72)

0.5

WBC count × 103 per µL

7.6 (1.9–25.8)

6.9 (1.7–48.9)

0.5

Platelet count × 103 per µL

585 (454–1741)

559 (14–1798)

0.7

PB blast%

–

0 (0–9)

<0.0001a

PB blasts ≥1%

–

9

<0.0001a

BM blast%

1 (0–4)

3 (0–8)

0.001a

BM blast ≥5%

–

8

<0.0001a

BM RS%

47.5 (15–90)

35 (15–80)

0.2

Thrombosis %

14 (18)

2 (8)

0.3

 Arterial

6 (43)

1 (50)

 

 Venous

6 (43)

1 (50)

Cytogenetics

Evaluable = 72

Evaluable = 24

 

Abnormal karyotype (except-Y)

11 (15)

4 (17)

0.9

Monosomal karyotype

2 (3)

2 (8)

0.3

Complex karyotype

2 (3)

2 (8)

0.3

IPSS cytogenetics

Good

63 (88)

20 (83)

0.7

Intermediate

4 (6)

1 (4)

Poor

5 (7)

3 (13)

IPSS-R cytogenetics

Very good

5 (7)

–

0.4

Good

59 (81)

20 (83)

Intermediate

4 (5)

1 (4)

Poor

3 (4)

1 (4)

Very poor

2 (3)

2 (8)

Pathogenic variants

 ASXL1

Evaluable = 49; 10 (20)

Evaluable = 17; 4 (24)

0.9

 DNMT3A

Evaluable = 43; 6 (14)

Evaluable = 15; 2 (13)

0.9

 TET2

Evaluable = 43; 3 (7)

Evaluable = 15; 2 (13)

0.5

 SF3B1

Evaluable = 111; 103 (93)

Evaluable = 17; 10 (59)

0.0005a

 SRSF2

Evaluable = 43; 1 (2)

Evaluable = 15; 2 (13)

0.1

 ZRSR2

Evaluable = 43; 1 (2)

Evaluable = 15; 0 (0)

0.4

 U2AF1

Evaluable = 43; 1 (2)

Evaluable = 15; 2 (13)

0.1

 JAK2 V617F

Evaluable = 43; 13 (30)

Evaluable = 15; 2 (13)

0.2

 CALR

Evaluable = 43; 2 (5)

Evaluable = 15; 0 (0)

0.3

 SETBP1

Evaluable = 40; 4 (10)

Evaluable = 15; 2 (13)

0.7

 TP53

Evaluable = 39; 1 (2)

Evaluable = 15; 0 (0)

0.4

Outcomes (evaluable = 183)

Median follow-up (months)

62 (45–80)

53 (18–123)

0.9

Leukemic transformation (%)

6 (4)

–

0.0003a

LFS (95% CI) years

Median not reached

Median not reached

0.4

OS (95% CI) years

6 (5–9)

7.3 (1.8–11.4)

0.3

  1. MDS/MPN-RS-T myelodysplastic/myeloproliferative neoplasms with RS and thrombocytosis, MDS/MPN-U myelodysplastic/myeloproliferative neoplasms, unclassifiable, Hb hemoglobin, BM bone marrow, RS ring sideroblast, IPSS International Prognostic Scoring System, IPSS-R Revised International Prognostic Scoring System, LFS acute myeloid leukemia-free survival, OS overall survival.
  2. aDenotes statistical significance (P < 0.05).
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