Rocchi E, Gibertini P, Cassanelli M, Pietrangelo A, Borghi A, Pantaleoni M, Jensen J, Ventura E
Br J Dermatol. 1986 May;114(5):621-9. doi: 10.1111/j.1365-2133.1986.tb04071.x.
Twenty-five patients with overt clinical and biochemical findings of porphyria cutanea tarda took part in a study comparing intensive phlebotomy with slow subcutaneous desferrioxamine treatment. Fifteen male patients (Group A) had intensive venesection therapy. Ten patients (Group B) with associated diseases (minor thalassemia, cardiovascular impairment, pulmonary tuberculosis or severe liver cirrhosis) received 1.5 g of desferrioxamine by slow subcutaneous infusion using an automatic syringe pump 5 days a week. No patient complained of appreciable side effects. Serum iron, ferritin and uroporphyrins were normalized in all subjects by the end of treatment. The mean time necessary for complete recovery was 13.8 months (range 9-19) and 11.2 months (range 6-14) in Groups A and B, respectively. Liver function significantly improved during and after the treatments in both groups. We conclude that recovery from porphyria cutanea tarda can be achieved equally well using phlebotomy or desferrioxamine subcutaneous infusion. Phlebotomy is easily performed and remains the treatment of choice; slow subcutaneous desferrioxamine treatment, although expensive, is recommended when severe associated diseases contra-indicate venesection.
25例有迟发性皮肤卟啉症明显临床和生化表现的患者参与了一项比较强化放血疗法与缓慢皮下注射去铁胺治疗的研究。15例男性患者(A组)接受强化静脉放血治疗。10例伴有相关疾病(轻度地中海贫血、心血管损害、肺结核或严重肝硬化)的患者(B组)每周5天使用自动注射泵通过缓慢皮下输注接受1.5 g去铁胺治疗。没有患者抱怨有明显副作用。治疗结束时,所有受试者的血清铁、铁蛋白和尿卟啉均恢复正常。A组和B组完全恢复所需的平均时间分别为13.8个月(范围9 - 19个月)和11.2个月(范围6 - 14个月)。两组治疗期间及治疗后肝功能均显著改善。我们得出结论,迟发性皮肤卟啉症采用放血疗法或皮下注射去铁胺均可取得同样良好的恢复效果。放血疗法操作简便,仍是首选治疗方法;缓慢皮下注射去铁胺治疗虽然昂贵,但在严重相关疾病禁忌静脉放血时推荐使用。