Recent studies have shown that angiogenesis plays an important role in the biology of hematological malignancies including essential thrombocythemia (ET). Using cytofluorimetric analysis, the levels of angiogenic factors, as well as the number of circulating endothelial cells (CECs), were determined in 65 patients with ET, including 33 previously untreated and 32 receiving cytoreductive therapy. Correlations between markers of angiogenesis and JAK2-V617F mutational status were also assessed. We found significantly higher levels of vascular endothelial growth factor (VEGF) and markedly decreased levels of placental growth factor in untreated patients with ET with respect to control subjects. VEGF levels were significantly increased in patients with white blood count >8.7 (x 10(9)/L) vs.