T. Barbui et al., TREATMENT STRATEGIES IN ESSENTIAL THROMBOCYTHEMIA - A CRITICAL-APPRAISAL OF VARIOUS EXPERIENCES IN DIFFERENT CENTERS, Leukemia & lymphoma, 22, 1996, pp. 149-160
The therapeutic strategy in patients with Essential Thrombocythemia (E
T) is a difficult balance between the prevention of bleeding and throm
botic complications and the risks of drug side effects and toxicity. M
ajor bleeding is rare and seem to be related to higher platelet counts
: therefore, a platelet count over 1500 x 10(9)/L is generally regarde
d as an indication for cytoreduction. Thrombotic complications include
microvascular occlusive symptoms, which are reversible with low-dose
aspirin, and large vessels thrombosis. The risk of major thrombosis is
higher in ET patients aged more than 60 ys. and with previous occlusi
ve event. In this high-risk group, the non-alkylating agent hydroxyure
a (HU) significantly reduces the rate of vascular complications and ha
s emerged as the treatment of choice. However, the long-term risk/bene
fit of HU remains disputed because its leukemogenic potential has not
been ruled out. This holds also for other myelosuppressive agents, suc
h as busulphan and pipobroman. Other drugs of particular interest for
young patients include recombinant alpha-interferon (IFN) and Anagreli
de. Both of them are effective in lowering platelet count, but their e
fficacy in reducing clinical complications remains to be demonstrated.
However, both IFN and Anagrelide have shown to have frequent and clin
ically important side effects. Thus, further clinical studies are requ
ired to establish their role in the strategy of ET patient treatment.