Taylor P C, Dolan G, Ng J P, Paul B, Collin R, Reilly J T
Rotherham District General, Nottingham.
Br J Haematol. 1996 Jan;92(1):55-9. doi: 10.1046/j.1365-2141.1996.00303.x.
The efficacy and tolerability of rIFN-alpha has been evaluated in 17 selected patients with symptomatic polycythaemia vera, diagnosed according to the PRV Study Group criteria. Complete disease control (CR) was achieved, after 1-12 months, in nine patients, with partial control in a further five cases. Three patients failed to respond. Pruritus significantly improved in 83% (10/12) of cases, following 1-28 weeks of treatment. Six patients (35%), however, were unable to tolerate rIFN-alpha, on account of weight loss, myalgia and mental changes. Overall, alpha-interferon therapy significantly improved venesection requirements, MCV and PCV values, platelet counts, pruritus scores and the degree of splenomegaly. Analysis of pooled published data (100 evaluable patients, including the present study) revealed an overall CR of 60%, a PR of 27%, and a failure rate of 13%. Significant pruritus control (> 50% improvement) occurred in 77% of cases. rIFN-alpha appears to be an effective therapy for PV-associated myeloproliferation and/or pruritus, although side-effects remain a concern. Long-term studies are now indicated to determine if the natural history of the disease is altered, in particular whether the incidence of myelofibrosis and/or leukaemic transformation is reduced.
根据真性红细胞增多症研究组标准，对17例有症状的真性红细胞增多症患者评估了重组干扰素α（rIFN-α）的疗效和耐受性。1至12个月后，9例患者实现了完全疾病控制（CR），另有5例部分控制。3例患者无反应。治疗1至28周后，83%（12例中的10例）的瘙痒症明显改善。然而，6例患者（35%）因体重减轻、肌痛和精神改变而无法耐受rIFN-α。总体而言，α干扰素治疗显著改善了放血需求、平均红细胞体积（MCV）和红细胞压积（PCV）值、血小板计数、瘙痒评分和脾肿大程度。对汇总的已发表数据（包括本研究在内的100例可评估患者）分析显示，总体CR率为60%，部分缓解（PR）率为27%，失败率为13%。77%的病例瘙痒得到显著控制（改善>50%）。rIFN-α似乎是治疗真性红细胞增多症相关骨髓增殖和/或瘙痒的有效疗法，尽管副作用仍是一个问题。现在需要进行长期研究以确定疾病的自然史是否改变，特别是骨髓纤维化和/或白血病转化的发生率是否降低。
