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[去铁胺成功治疗血液透析相关迟发性皮肤卟啉症]

[Successful treatment of haemodialysis-related porphyria cutanea tarda with deferoxamine].

作者信息

Pitche P, Corrin E, Wolkenstein P, Revuz J, Bagot M

机构信息

Service de Dermatologie, CHU Henri Mondor, 51, avenue du Maréchal de Lattre de Tassigny, 94010 Créteil.

出版信息

Ann Dermatol Venereol. 2003 Jan;130(1 Pt 1):37-9.

Abstract

BACKGROUND

End-stage renal failure and long-term hemodialysis treatment promote porphyria cutanea tarda. Iron overload is often associated with this disease and is thought to play a role in its pathogenesis. We report a case of hemodialysis related-porphyria cutanea tarda improved by deferoxamine.

CASE REPORT

A 45-year-old man, with end-stage renal failure and who had received hemodialysis treatment since 1993, presented a several months-history of blisters of the face and the dorsum of the hands. Laboratory analysis showed: hemoglobin 10 g/dl; a moderate hepatic cytolysis; ferritin 195 ng/l. HIV, HBV, HCV serologies were negative. Porphyries analyses showed a porphyria cutanea tarda pattern. The cutaneous histology was non specific; direct immunofluorescence was negative. The patient received deferoxamine (40 mg/kg intravenously every week for 6 weeks) which led to dramatic improvement of the symptoms.

DISCUSSION

Several treatments are proposed in the management of dialysis-related porphyria cutanea tarda. This case confirms that deferoxamine can induce rapid and prolonged remission.

摘要

背景

终末期肾衰竭和长期血液透析治疗会促使迟发性皮肤卟啉症的发生。铁过载常与该疾病相关,并被认为在其发病机制中起作用。我们报告一例经去铁胺治疗后病情改善的血液透析相关性迟发性皮肤卟啉症病例。

病例报告

一名45岁男性,患有终末期肾衰竭,自1993年起接受血液透析治疗,出现面部和手背水疱数月。实验室分析显示:血红蛋白10g/dl;中度肝细胞溶解;铁蛋白195ng/l。HIV、HBV、HCV血清学检查均为阴性。卟啉分析显示为迟发性皮肤卟啉症模式。皮肤组织学检查无特异性;直接免疫荧光检查为阴性。患者接受去铁胺治疗(每周静脉注射40mg/kg,共6周),症状得到显著改善。

讨论

在透析相关性迟发性皮肤卟啉症的治疗中提出了几种治疗方法。该病例证实去铁胺可诱导快速且持久的缓解。

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