Department of Medical Oncology, Institute Jules Bordet, Brussels, Belgium.
Eur J Clin Invest. 2010 Sep;40(9):828-34. doi: 10.1111/j.1365-2362.2010.02334.x. Epub 2010 Jul 1.
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.
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.
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.
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.
瘙痒是真性红细胞增多症（PV）的一个特征性表现，大约 40%的患者会出现这种症状。在大多数情况下，瘙痒的特征是在任何温度下接触水（水诱发性）时触发。
对从 1965 年至今发表的所有与 PV、PV 相关瘙痒和水诱发性瘙痒相关的英文文献进行了详细的 MEDLINE 检索。
在过去几十年中，已经尝试了许多不同的治疗选择，包括抗组胺药、抗抑郁药、干扰素α、放血疗法、光疗、铁补充剂和骨髓抑制药物，但均显示出混合结果。最近，靶向 JAK2 和哺乳动物雷帕霉素靶蛋白（mTOR）的药物已显示出令人印象深刻的临床获益。
PV 相关瘙痒是 PV 患者发病率的主要原因。干扰血清素摄取的抗抑郁药物有一定疗效。细胞减少疗法保留用于难治性病例。针对 JAK2 和 mTOR 抑制剂的靶向治疗带来了新的希望。
