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伴有网织红细胞减少的难治性特发性自身免疫性溶血性贫血

Hard-To-Treat Idiopathic Refractory Autoimmune Haemolytic Anaemia with Reticulocytopenia.

作者信息

Aveiro Marcelo, Ferreira Gisela, Matias Carla, Oliveira Ana, Rodrigues Tatiana

机构信息

Internal Medicine Department, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal.

Haematology Department, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal.

出版信息

Eur J Case Rep Intern Med. 2020 Dec 2;7(12):002112. doi: 10.12890/2020_002112. eCollection 2020.

Abstract

UNLABELLED

Autoimmune haemolytic anaemia (AIHA) is an uncommon condition characterized by increased destruction of erythrocytes associated with reticulocytosis in the great majority of cases. We present the case of a 68-year-old woman with jaundice and malaise. Investigation revealed AIHA with reticulocytopenia. The patient failed to respond to prednisolone or to rituximab. Azathioprine and epoetin beta were subsequently started, the prednisolone dose was increased, and the patient began to respond after 1 month. In AIHA, reticulocytopenia is a very rare presentation and a sign of great severity and poor outcome. The scarcity of therapeutic options in refractory cases poses a major challenge for physicians.

LEARNING POINTS

Autoimmune haemolytic anaemia is a rare disorder characterized by decompensated acquired haemolysis caused by the host's immune system acting against its own red cell antigens.Concomitant presentation with reticulocytopenia is very rare and a sign of great severity and poor outcome.Treatment options in refractory cases still greatly rely on individual experience and expert opinion.

摘要

未标注

自身免疫性溶血性贫血(AIHA)是一种不常见的疾病,在大多数情况下,其特征是红细胞破坏增加并伴有网织红细胞增多。我们报告一例68岁女性,有黄疸和全身不适症状。检查发现为伴有网织红细胞减少的AIHA。该患者对泼尼松龙或利妥昔单抗均无反应。随后开始使用硫唑嘌呤和促红细胞生成素β,增加泼尼松龙剂量,1个月后患者开始出现反应。在AIHA中,网织红细胞减少是一种非常罕见的表现,是病情严重且预后不良的标志。难治性病例中治疗选择匮乏给医生带来了重大挑战。

学习要点

自身免疫性溶血性贫血是一种罕见疾病,其特征是宿主免疫系统针对自身红细胞抗原导致的失代偿性获得性溶血。伴有网织红细胞减少的情况非常罕见,是病情严重且预后不良的标志。难治性病例的治疗选择仍很大程度上依赖个人经验和专家意见。

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本文引用的文献

1
Azathioprine-Induced Rapid Hepatotoxicity.硫唑嘌呤诱导的快速肝毒性。
J Pharm Technol. 2014 Feb;30(1):18-20. doi: 10.1177/8755122513504078. Epub 2014 Jan 6.
2
How I treat autoimmune hemolytic anemia.我如何治疗自身免疫性溶血性贫血。
Blood. 2017 Jun 1;129(22):2971-2979. doi: 10.1182/blood-2016-11-693689. Epub 2017 Mar 30.
10
Azathioprine.
Clin Rheum Dis. 1984 Aug;10(2):325-32.

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