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尽管定期使用去铁胺仍发生输血性血色素沉着症:病例报告

Transfusion haemosiderosis inspite of regular use of desferrioxamine: case report.

作者信息

Mwanda O W, Otieno C F, Abdalla F K

机构信息

Department of Haematology and Blood Transfussion, College of Health Sciences, University of Nairobi, PO Box 19676, Nairobi, Kenya.

出版信息

East Afr Med J. 2004 Jun;81(6):326-8. doi: 10.4314/eamj.v81i6.9184.

Abstract

We describe a case of a female who developed haemosiderosis, in the course of treatment for very severe unstable aplastic anaemia for fourteen years. She was 37 years old at the time of initial diagnosis. Her management consisted of regular blood transfusions aimed at haemoglobin above 8.5 g/dl, antimicrobials, oxymetholone, low dose prednisone and folate. She had received about seventy five units of blood at the start of 2 grams of desferrioxamine with every subsequent blood transfusion. Annual tests of serum ferritin showed progressive increase. She developed skin changes, diabetes mellitus, heart disease, recurrent infections, generalized joint and abdominal pains and liver failure. She died within six weeks of developing congestive heart failure coupled with liver failure due to haemosiderosis despite regular use of desferrioxamine.

摘要

我们描述了一例女性患者,她在治疗极重型不稳定型再生障碍性贫血的过程中患了含铁血黄素沉着症,病程长达14年。初次诊断时她37岁。她的治疗包括定期输血以使血红蛋白维持在8.5 g/dl以上、使用抗菌药物、羟甲烯龙、低剂量泼尼松和叶酸。在开始使用去铁胺时,她已接受了约75单位的血液输注,此后每次输血都同时使用2克去铁胺。血清铁蛋白的年度检测显示其逐渐升高。她出现了皮肤改变、糖尿病、心脏病、反复感染、全身性关节和腹痛以及肝功能衰竭。尽管定期使用去铁胺,但她在因含铁血黄素沉着症并发充血性心力衰竭和肝功能衰竭后的六周内死亡。

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