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一名腺苷脱氨酶缺乏-重症联合免疫缺陷患者患伯基特淋巴瘤,接受聚乙二醇化腺苷脱氨酶治疗。

Burkitt's lymphoma in a patient with adenosine deaminase deficiency-severe combined immunodeficiency treated with polyethylene glycol-adenosine deaminase.

作者信息

Husain Maitham, Grunebaum Eyal, Naqvi Ahmed, Atkinson Adelle, Ngan Bo-Yee, Aiuti Alessandro, Roifman Chaim M

机构信息

Division of Immunology/Allergy, The Hospital for Sick Children and The University of Toronto, Toronto, Ontario, Canada.

出版信息

J Pediatr. 2007 Jul;151(1):93-5. doi: 10.1016/j.jpeds.2007.03.059.

Abstract

We describe a patient with severe combined immunodeficiency because of aberrations in adenosine deaminase (ADA) who despite adequate replacement with polyethylene glycol-linked ADA (PEG-ADA) for 13 years developed Burkitt's lymphoma. Although treatment corrected the metabolic abnormalities caused by ADA deficiency, it failed to fully restore cellular immunity.

摘要

我们描述了一名因腺苷脱氨酶(ADA)异常而患有严重联合免疫缺陷的患者,尽管用聚乙二醇连接的ADA(PEG-ADA)进行了13年的充分替代治疗,但仍发生了伯基特淋巴瘤。虽然治疗纠正了由ADA缺乏引起的代谢异常,但未能完全恢复细胞免疫。

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