Table 3.
Comparison of pediatric ET cases as reported by Italian Association for Pediatric Haematology Oncology Italian (AIEOP) series [15] and by a systematic review [43]. Plts = platelets number, pts = patients. (m = month, y = year).
| AIEOP Series [15] |
Review [43] |
|
|---|---|---|
| # of patients F/M |
89 1.8/1 (65%) |
396 1.3/1 (58%) |
| Median age (range) |
7 years (6 m–17.5 y) |
9 years (6 m–20 y) |
| Plts × 109/L | 503–4400 | 450–4500 |
| Microvascular symptoms | 30% | 23% |
| Splenomegaly | 38% | 55% |
| Hemorrhagies | 9% minor | 4.8% |
| Thrombosis | 3.3% (clonal ET) | 4% (venous > arterial) |
| Transformation | No | 2% (MF) |
| Clonal cases (%) | 23/89 (25.8%) | 388 (40%) |
|
JAK2V617F + Allele burden (%) |
14 (15%) 26.2 |
130 (33%) 24.2 |
| MPLW515L (56 pts tested) | 1 | 4 (1%) |
| CALR mutations (74 pts tested) | 6 (6.5%) | 23 (6%) |
| X-CIP test pos (23 females tested) | 6 (2 JAK2V617F; 2 CALR pts) |
Not known |
| Marrow Biopsy (done) | 45 (50%) | 52% |