Main clinical findings of children with essential thrombocythemia
Patient no. . | Sex . | Clonal/JAK2 . | Age at diagnosis, y . | Age at analysis, y . | Signs and symptoms . | Treatment . |
|---|---|---|---|---|---|---|
| 1 | F | Yes/V617F | 0, 8 | 9 | Budd-Chiari syndrome and inferior vena cava thrombosis | OLT + warfarin |
| 2 | F | No/WT | 2 | 2 | None | None |
| 3 | F | No/WT | 3 | 14 | None | ASA |
| 4 | F | No/WT | 4 | 6 | None | None |
| 5 | M | WT | 4 | 15 | None | ANAGR |
| 6 | F | No/WT | 6 | 7 | Epistaxis, headache | ASA |
| 7 | M | WT | 6 | 7 | None | ANAGR |
| 8 | M | V617F | 6 | 7 | Headache | ASA |
| 9 | F | Yes/WT | 7 | 8 | None | ASA + ANAGR |
| 10 | F | No/WT | 7 | 11 | Headache | ANAGR |
| 11 | F | No/WT | 8 | 9 | None | ANAGR |
| 12 | F | No/WT | 8 | 20 | Headache | IFN/ASA |
| 13 | F | No/WT | 8 | 31 | None | ASA |
| 14 | M | V617F | 9 | 15 | None | ANAGR |
| 15 | F | No/WT | 11 | 15 | Headache | ANAGR |
| 16 | F | No/WT | 12 | 12 | None | ASA |
| 17 | M | WT | 12 | 24 | Headache | ASA |
| 18 | F | Yes/WT | 13 | 16 | None | None |
| 19 | F | No/WT | 13 | 18 | Headache | ASA |
| 20 | F | Yes/V617F | 14 | 15 | None | ANAGR |
Patient no. . | Sex . | Clonal/JAK2 . | Age at diagnosis, y . | Age at analysis, y . | Signs and symptoms . | Treatment . |
|---|---|---|---|---|---|---|
| 1 | F | Yes/V617F | 0, 8 | 9 | Budd-Chiari syndrome and inferior vena cava thrombosis | OLT + warfarin |
| 2 | F | No/WT | 2 | 2 | None | None |
| 3 | F | No/WT | 3 | 14 | None | ASA |
| 4 | F | No/WT | 4 | 6 | None | None |
| 5 | M | WT | 4 | 15 | None | ANAGR |
| 6 | F | No/WT | 6 | 7 | Epistaxis, headache | ASA |
| 7 | M | WT | 6 | 7 | None | ANAGR |
| 8 | M | V617F | 6 | 7 | Headache | ASA |
| 9 | F | Yes/WT | 7 | 8 | None | ASA + ANAGR |
| 10 | F | No/WT | 7 | 11 | Headache | ANAGR |
| 11 | F | No/WT | 8 | 9 | None | ANAGR |
| 12 | F | No/WT | 8 | 20 | Headache | IFN/ASA |
| 13 | F | No/WT | 8 | 31 | None | ASA |
| 14 | M | V617F | 9 | 15 | None | ANAGR |
| 15 | F | No/WT | 11 | 15 | Headache | ANAGR |
| 16 | F | No/WT | 12 | 12 | None | ASA |
| 17 | M | WT | 12 | 24 | Headache | ASA |
| 18 | F | Yes/WT | 13 | 16 | None | None |
| 19 | F | No/WT | 13 | 18 | Headache | ASA |
| 20 | F | Yes/V617F | 14 | 15 | None | ANAGR |
The children were followed in 7 different pediatric units. The median clinical follow up was 7.5 years (range, 18 months to 24 years). The age of patients at diagnosis and at the moment of the analyses is reported. Treatments were chosen by the referring physicians, upon local criteria. Patient no. 1 underwent orthotopic liver transplantation (OLT) at 7 years of age for Budd-Chiari syndrome presented at diagnosis. Patient no. 12 was initially treated with interferon alpha (IFN) with partially transitory response and then shifted to low-dose aspirin (ASA). Patients nos. 16 and 20 were treated with anagrelide (ANAGR) for only 6 months each. No relation between age and molecular findings is recognizable