Myeloproliferative Disorders

PRV is generally treated with phlebotomy with or without cytoreductive therapy, usually hydroxyurea. Low-dose aspirin also has benefits in terms of a reduction in the risk of thrombosis. ET is also managed with cytoreductive therapy in selected patients. Treatment is beneficial in high-risk patients, defined as those with extreme ET or those with cardiovascular or cerebrovascular risk factors. Patients with a prior history of thrombosis and those over 60 years of age should be treated (60). The current therapy of choice is hydroxyurea. An alternative is anagrelide, the specific megakaryocyte inhibitor. A recent randomized trial demonstrated a higher incidence of arterial thrombosis and a higher incidence of progression to chronic idiopathic MF with anagrelide when compared to hydroxyurea (61). Chronic idiopathic MF is managed with supportive care measures. Androgens and erythropoietin may be beneficial for the management of anemia. Splenectomy may be beneficial in selected patients but carries a high risk of morbidity and mortality and can result in transfusion independence. Thalidomide may also be beneficial in chronic MF (62). The only curative therapy is an allogeneic bone marrow stem-cell transplant, but this treatment has limited applicability given the advanced age of most patients and limited availability of a human leucocyte antigen-matched sibling donor. The introduction of reduced intensity regimens for preparation prior to transplant has opened up this option to a greater number of patients (63).

CML is currently managed with imatinib, the tyrosine kinase inhibitor. This is associated with very high rates of complete hematological response and also cytogenetic and molecular responses (64). Newer tyrosine kinase inhibitors that are effective in patients resistant to imatinib are in development. For suitable candidates who do not respond adequately to imatinib, an allogeneic transplant remains the only known curative option for this disorder.

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