Background: Pruritus is a defining feature of polycythemia vera (PV) and is seen in approximately 40% of patients. In most cases, the pruritus is characteristically triggered by contact with water (aquagenic) at any temperature.
Materials and methods: A detailed MEDLINE search for all English language articles related to PV, PV-associated pruritus and aquagenic pruritus that were published from 1965 till date was carried out.
Results: Many different treatment options have been tried over the past several decades, including antihistamines, antidepressants, interferon alpha, phlebotomy, phototherapy, iron supplements and myelosuppressive medications, all demonstrating mixed results. Recently, agents that target JAK2 and mammalian target of rapamycin (mTOR) have shown impressive clinical benefit.
Conclusion: PV-associated pruritus is a major cause of morbidity amongst patients with PV. Antidepressant medications interfering with serotonin uptake are somewhat efficacious. Cytoreductive therapy is reserved for refractory cases. Targeted therapy with JAK2 and mTOR inhibitors offers renewed hope.