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迟发性皮肤卟啉症导致肾透析患者出现继发性含铁血黄素沉着症:一例报告

Secondary hemosiderosis presented by porphyria cutanea tarda in a kidney dialysis patient: A case report.

作者信息

AlGahtani Farjah H, Stuckey Ruth, Alqahtany Fatima S

机构信息

Hematology-Oncology Division, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia.

Hematology Department, Hospital Universitario de Gran Canaria Dr. Negrín, Las Palmas, Spain.

出版信息

SAGE Open Med Case Rep. 2020 Feb 22;8:2050313X20907815. doi: 10.1177/2050313X20907815. eCollection 2020.

Abstract

A 68-year-old woman with chronic kidney disease receiving dialysis and iron supplementation presented to our hospital with painful blisters, fragile skin, and changes to skin pigmentation on the dorsal side of both upper and lower limbs. Skin biopsy findings and an increase in urine porphyrins confirmed the diagnosis of porphyria cutanea tarda. Upon examination, extremely high serum ferritin levels (6000 µg/L) suggested iron overload. Oral iron supplementation was immediately discontinued, and the patient received treatment with the iron chelators deferoxamine, 10 mg/kg/day intravenously for 4 days, and deferasirox, 540 mg/day orally. After a 4-month follow-up, ferritin levels were normal (97.7 µg/L) and the cutaneous manifestations of porphyria cutanea tarda had improved. Complete remission has been maintained for the last 2 years, and the patient's liver and heart function are normal. This case of porphyria cutanea tarda caused by secondary hemosiderosis highlights the potential toxicity of iron accumulation as a result of excessive iron supplementation. Although not approved for the treatment of patients on hemodialysis, we report the efficacy of deferasirox without any adverse effects in this case. We also stress the importance of the close monitoring of serum iron levels in kidney dialysis-and indeed all iron-supplemented-patients to avoid potential hepatic, cardiac, and endocrine damage.

摘要

一名68岁患有慢性肾脏病且正在接受透析和铁剂补充治疗的女性因双上肢和双下肢背侧出现疼痛性水疱、皮肤脆弱及皮肤色素沉着改变前来我院就诊。皮肤活检结果及尿卟啉增加确诊为迟发性皮肤卟啉病。检查时,极高的血清铁蛋白水平(6000µg/L)提示铁过载。立即停用口服铁剂补充,患者接受铁螯合剂去铁胺治疗,静脉注射10mg/kg/天,共4天,及地拉罗司治疗,口服540mg/天。经过4个月的随访,铁蛋白水平恢复正常(97.7µg/L),迟发性皮肤卟啉病的皮肤表现有所改善。在过去2年中一直保持完全缓解,患者的肝脏和心脏功能正常。这例由继发性含铁血黄素沉着症引起的迟发性皮肤卟啉病病例凸显了过度补充铁剂导致铁蓄积的潜在毒性。尽管地拉罗司未被批准用于血液透析患者的治疗,但我们报告了其在本例中的疗效且无任何不良反应。我们还强调密切监测肾脏透析患者以及所有补充铁剂患者血清铁水平的重要性,以避免潜在的肝脏、心脏和内分泌损害。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e8eb/7036493/e6f793f3deda/10.1177_2050313X20907815-fig1.jpg

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