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一名患有克里斯马斯病的儿童体内IgA抗因子IX抑制剂的自发消失

Spontaneous disappearance of an IgA anti-factor IX inhibitor in a child with Christmas disease.

作者信息

Carroll R R, Panush R S, Kitchens C S

出版信息

Am J Hematol. 1984 Oct;17(3):321-5. doi: 10.1002/ajh.2830170312.

DOI:10.1002/ajh.2830170312
PMID:6475941
Abstract

The few inhibitors to blood coagulation factor IX in patients with Christmas disease (hemophilia B) that have been studied have been shown to belong to the IgG class of immunoglobulins. We report the first case in which a factor IX inhibitor was of the IgA class. Additionally, he appears to be the youngest patient with hemophilia B to have developed an inhibitor. His inhibitor complicated treatment of the patient for several years because of its anamnestic rise following factor IX concentrate administration. It then spontaneously vanished and has not returned in spite of repeated factor IX complex administration.

摘要

在已研究的患有克里斯马斯病(血友病B)的患者中,少数凝血因子IX抑制剂已被证明属于免疫球蛋白IgG类。我们报告了首例凝血因子IX抑制剂为IgA类的病例。此外,他似乎是患血友病B并产生抑制剂的最年轻患者。由于其在给予凝血因子IX浓缩物后呈记忆性升高,该抑制剂使患者的治疗复杂化了数年。随后它自行消失,尽管反复给予凝血因子IX复合物,它也未再出现。

相似文献

1
Spontaneous disappearance of an IgA anti-factor IX inhibitor in a child with Christmas disease.一名患有克里斯马斯病的儿童体内IgA抗因子IX抑制剂的自发消失
Am J Hematol. 1984 Oct;17(3):321-5. doi: 10.1002/ajh.2830170312.
2
Studies on Christmas disease: investigation and treatment of a familial acquired inhibitor of factor IX.关于克里斯马斯病的研究:一种家族性获得性IX因子抑制剂的调查与治疗
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Survey of the anti-factor IX immunoglobulin profiles in patients with hemophilia B using a fluorescence-based immunoassay.使用基于荧光的免疫测定法对乙型血友病患者的抗因子IX免疫球蛋白谱进行调查。
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Characterization of an occult inhibitor to factor IX in a haemophilia B patient.一名B型血友病患者中IX因子隐匿性抑制剂的特征分析
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引用本文的文献

1
Laboratory testing for factor VIII and IX inhibitors in haemophilia: A review.实验室检测血友病患者的因子 VIII 和 IX 抑制剂:综述。
Haemophilia. 2018 Mar;24(2):186-197. doi: 10.1111/hae.13424. Epub 2018 Feb 15.