Table 2 Clinical characteristics, molecular analysis and constitutional symptoms of patients with ET at presentation and treatment according to applied diagnostic criteria

From: Clinical impact of bone marrow morphology for the diagnosis of essential thrombocythemia: comparison between the BCSH and the WHO criteria

 

BCSH-defined ET (criteria A1-A3) 1, 2

WHO-defined ET criteria 3

P-value

General characteristics

n

238

232

 

 Age at diagnosis (years)

61.3 (18.8–88.8)

57.2 (17.5–88.8)

0.073

 Sex male/female

86/123

93/139

0.750

Clinical characteristics a

 Platelets (× 109/l)

769 (452–2530)

754 (450–2490)

0.539

 Hemoglobin (g/dl)

14.2 (8.6–17.3)

14.4 (8.6–17.3)

0.826

 Hematocrit (%)

42.9 (42.9–52.0)

42.7 (29.9–52.6)

0.630

 WBC (× 109/l)

9.4 (2.21–31.32)

8.82 (2.21–22.3)

0.057

 LDH (U/l)

221 (118–763)

207 (104–763)

<0.001

 Palpable splenomegaly (218/238)b

16.4% (39)

11.9% (26)

0.183

 Fibrosis grading 1

8.4% (20)

0.0% (0)

<0.001

Molecular characteristics

 Pathogenetic mutation present (169/238)b

100% (238)

72.8% (169)

JAK2 V617F (220/238)b

72.7% (173)

80.5% (136)

0.016

CALR (141/181)b

32.0% (58)

16.0% (27)

0.011

MPL (53/75)b

9.3% (7)

3.6% (6)

0.771

Symptoms at diagnosis

 Constitutional symptoms (169/200)b

16.0% (32)

14.8% (25)

0.774

Weight loss

4.5% (9)

4.1% (7)

1.000

Night sweats

8.5% (17)

8.3% (14)

1.000

Fatigue

5.0% (10)

5.9% (10)

0.818

 Pruritus (175/202)b

2.0% (4)

2.3% (4)

1.000

Cytoreductive Therapy (164/191) b

 Hydroxurea

42.9% (82)

42.1% (69)

0.494

 Interferon-alpha

34.6% (66)

30.5% (50)

0.429

 Anagrelide

30.4% (58)

34.1% (56)

0.494

 JAK1/2-Inhibitor

4.7% (9)

3.0% (5)

0.586

 Busulfan

2.6% (5)

2.4% (4)

1.000

 Othersc

4.2% (8)

0.6% (1)

0.042

Antithrombotic therapy with low dose aspirin (160/189)b

90.5% (171)

88.8% (142)

0.602

  1. Abbreviations: BCSH, British Committee of Standards in Haematology; CALR, calreticulin exon 9 mutations; ET, essential thrombocythemia; JAK2, Janus kinase 2; LDH, serum lactate dehydrogenase; MPL, myeloproliferative leukemia oncogene; WBC, white blood cell count.
  2. aMedian, range.
  3. bNumber evaluable in each cohort.
  4. cPipobroman, P32 and other cytoreductive agents.