Table 2. Clinical characteristics, molecular analysis and constitutional symptoms of patients with ET at presentation and treatment according to applied diagnostic criteria.
| BCSH-defined ET (criteria A1-A3)1, 2 | WHO-defined ET criteria3 | 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 characteristicsa | |||
| 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 |
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.
a
Median, range.
b
Number evaluable in each cohort.
c
Pipobroman, P32 and other cytoreductive agents.