Table 1 Patient characteristics, for the entire cohort, for ET and PV patients.

From: Impact of treatment for adolescent and young adults with essential thrombocythemia and polycythemia vera

 

ALL (n = 348)

ET (n = 278)

PV (n = 70)

p-value

 

n

%

n

%

n

%

 

Age at diagnosis (median, IQR/range)

20yo

18–23/2–25

21yo

18–23/2–25

20yo

16–23/2–25

0.320

Sex

      

<0.001

Male

99

28.4

62

22.3

37

52.9

 

Female

249

71.6

216

77.7

33

47.1

 

Mutational status

      

<0.001

JAK2

217

62.4

147

52.9

70

100.0

 

Exon 14

212

 

147

 

65

  

Allele burden (median, IQR) (data available for 58 pts)

22.4

14–22

15.8

10.7–25.5

32.5

22.5–44

<0.001

Exon 12

5

 

0

 

5

  

CALR

43

12.3

43

15.5

0

0.0

 

Type 1

14

 

14

 

0

  

Type 2

7

 

7

 

0

  

Unknown

22

 

22

 

0

  

MPL

3

0.9

3

1.1

0

0.0

 

TN

85

24.4

85

30.5

0

0.0

 

Biochemical analysis

       

Hemoglobin (g/L) (median, IQR) (data available for 288 pts)

141.5

131–153

139

130–143

170

134–240

<0.001

Hematocrit (%) (median, IQR) (data available for 269 pts)

42.9

39.3–45.7

41.5

39–44

52

48.3–57.4

<0.001

Hematocrit >45% (data available for 269 pts)

84

31.2

41

18.4

43

93.5

<0.001

White blood cell (×109/ul) (median, IQR) (data available for 288 pts)

9

7.5–11.2

8.8

7.4–10.7

11.4

7.1–13.3

0.001

White blood cell >11 ×109/ul (data available for 288 pts)

77

26.7

52

21.5

25

54.3

<0.001

Platelets (×109/ul) (median, IQR) (data available for 293 pts)

874

621–1253

913

664–1304

628

355–919

<0.001

Platelet count >1000 ×109/ul (data available for 293 pts)

106

36.2

99

40.4

7

14.6

<0.001

LDH above normal range (data available for 217 pts)

98

45.2

73

42

25

58.1

0.056

Splenomegaly by palpation (data available for 305 pts)

54

17.7

33

13.1

21

38.9

<0.001

Reason for consulting

      

<0.001

Thrombotic event

27

7.8

13

4.7

14

20.0

 

Hemorrhage

0

0.0

0

0.0

0

0.0

 

MPN-related symptoms

72

20.7

57

20.5

15

21.4

 

Abnormal blood count

71

20.4

66

23.7

5

7.2

 

Abnormal blood count during pregnancy

15

4.3

12

4.3

3

4.3

 

Unknown

163

46.8

130

46.8

33

47.1

 

MPN-related symptoms

       

Plethoric face (data available for 296 pts)

10

3.4

0

0.0

10

19.6

<0.001

Pruritus (data available for 304 pts)

16

5.3

7

2.8

9

16.1

<0.001

Hyperviscosity (data available for 296 pts)

101

35.3

78

33.8

23

41.8

0.261

Constitutive symptoms (data available for 299 pts)

14

4.0

10

3.6

4

5.7

0.088

Fatigue (data available for 281 pts)

55

19.6

38

16.3

17

35.4

0.002

Sweets (data available for 281 pts)

5

1.8

1

0.4

4

8.3

<0.001

Vascular symptoms (data available for 318 pts)

34

10.7

30

11.6

4

6.7

0.263

CVRF (presence of any) (data available for 341 pts)

46

13.5

35

12.8

11

16.2

0.469

Smoking

32

9.4

26

9.5

6

8.8

0.859

High blood pressure

7

2.1

4

1.5

3

4.4

0.145

Dyslipidemia

1

0.3

1

0.4

0

0.0

1.000

Diabetes

2

0.6

0

0.0

2

2.9

0.039

Obesity

5

1.5

4

1.5

1

1.5

1.000

Thrombosis history (data available for 347 pts)

43

12.4

26

9.4

17

24.6

<0.001

Venous

31

8.9

21

7.6

10

14.5

0.070

BCS

11

3.2

6

2.2

5

7.2

 

BCS/CVT

1

0.3

0

0.0

1

1.4

 

CVT

10

2.9

7

2.5

3

4.3

 

DVT

3

0.9

3

1.1

0

0.0

 

PE

2

0.6

2

0.7

0

0.0

 

PVT

2

0.6

1

0.4

1

1.4

 

RVT

1

0.3

1

0.4

0

0.0

 

SpVT

1

0.3

1

0.4

0

0.0

 

Arterial

9

2.6

3

1.1

6

8.7

0.003

AMI

3

0.9

1

0.4

2

2.9

 

Stroke

2

0.6

1

0.4

1

1.4

 

TIA

4

1.2

1

0.4

3

4.3

 

Unknown localisation

3

0.9

2

0.7

1

1.4

 

Bleeding history (data available for 347 pts)

20

5.8

17

6.1

3

4.3

0.775

ELN risk

      

<0.001

Low

304

87.6

252

90.6

52

75.4

 

High

43

12.4

26

9.4

17

24.6

 

IPSET thrombosis (data available for 269 pts)

       

Low

N/A

 

122

45.4

N/A

  

Intermediate

N/A

 

110

40.9

N/A

  

High

N/A

 

37

13.7

N/A

  
  1. ALL entire cohort, AMI acute myocardial infarction, BCS Budd-Chiari syndrome, CVRF cardiovascular risk factors, CVT cerebral vein thrombosis, DVT deep vein thrombosis, ELN European LeukemiaNet, ET essential thrombocythemia, IPSET International prognostic score of thrombosis for essential thrombocythemia, IQR interquartile range, MPN myeloproliferative neoplasms, N/A not applicable, PE pulmonary embolism, pts patients, PV polycythemia vera, PVT portal vein thrombosis, RVT renal vein thrombosis, SpVT splenic vein thrombosis, TIA transient ischemic attack, TN triple negative