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血浆置换后纯红细胞再生障碍性贫血完全缓解。

Complete remission in pure red cell aplasia after plasmapheresis.

作者信息

Freund L G, Hippe E, Strandgaard S, Pelus L M, Erslev A J

出版信息

Scand J Haematol. 1985 Sep;35(3):315-8. doi: 10.1111/j.1600-0609.1985.tb01711.x.

DOI:10.1111/j.1600-0609.1985.tb01711.x
PMID:3933106
Abstract

A 17-year-old girl with a severe pure red cell aplasia (PRCA) and an appropriate elevation of erythropoietin titres was treated successfully with plasmapheresis, during which plasma was exchanged with human albumin and Ringer lactate. Before this treatment, therapeutic attempts with prednisolone, oxymetholone and infusions with fresh frozen plasma had all been without effect. Bone marrow culture studies revealed an inhibitory activity against BFU-E in the plasma, an activity which could not be demonstrated after the plasmapheresis. 22 months after the plasmapheresis the patient is still healthy without any medication. This case adds additional evidence for the presence of a pathogenetic erythroid inhibitory factor in the plasma of some patients with PRCA.

摘要

一名17岁患有严重纯红细胞再生障碍性贫血(PRCA)且促红细胞生成素水平适当升高的女孩,通过血浆置换成功治愈,在此过程中血浆与人白蛋白和乳酸林格液进行了交换。在该治疗之前,使用泼尼松龙、羟甲烯龙以及输注新鲜冰冻血浆的治疗尝试均无效。骨髓培养研究显示血浆中存在对爆式红系集落形成单位(BFU-E)的抑制活性,而血浆置换后这种活性未被检测到。血浆置换22个月后,患者未服用任何药物,仍然健康。该病例为某些PRCA患者血浆中存在致病性红系抑制因子提供了更多证据。

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Complete remission in pure red cell aplasia after plasmapheresis.血浆置换后纯红细胞再生障碍性贫血完全缓解。
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引用本文的文献

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[Chinese expert consensus on the diagnosis and treatment of acquired pure red cell aplasia (2020)].《获得性纯红细胞再生障碍性贫血诊断与治疗中国专家共识(2020年版)》
Zhonghua Xue Ye Xue Za Zhi. 2020 Mar 14;41(3):177-184. doi: 10.3760/cma.j.issn.0253-2727.2020.03.001.
2
[How I diagnose and treat pure red cell aplasia].[我如何诊断和治疗纯红细胞再生障碍性贫血]
Zhonghua Xue Ye Xue Za Zhi. 2020 Jan 14;41(1):5-9. doi: 10.3760/cma.j.issn.0253-2727.2020.01.002.
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Pure red cell aplasia.纯红细胞再生障碍性贫血。
Hematology Am Soc Hematol Educ Program. 2016 Dec 2;2016(1):51-56. doi: 10.1182/asheducation-2016.1.51.
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A case of pure red cell aplasia: follow-up on different immunosuppressive regimens.
Clin Investig. 1994 Dec;72(12):1051-5. doi: 10.1007/BF00577754.