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威斯科特-奥尔德里奇综合征,存活18年。用转移因子治疗。

Wiskott-Aldrich syndrome with 18-year survival. Treatment with transfer factor.

作者信息

Sellars W A, South M A

出版信息

Am J Dis Child. 1975 May;129(5):622-7. doi: 10.1001/archpedi.1975.02120420058019.

Abstract

An 18-year-old boy with Wiskott-Aldrich syndrome has severe symptoms of thrombocytopenia, recurrent infections, and atopic eczema. We believe he is the fifth oldest patient described with Wiskott-Aldrich syndrome. Recently, a malignant lymphoma of the histiocytic type appeared in the skin, while he was receiving transfer factor. To our knowledge, he is the only reported patient with lymphoma in the skin, but four other patients with Wiskott-Aldrich syndrome have developed malignant lymphoreticular lymphoma during transfer factor therapy. Detailed immunologic studies show failure to make a sustained antibody response to various antigens, lack of delayed hypersensitivity responsiveness, and failure of proliferative response to antigens in in vitro cultures. The IgE and IgA levels were high, and the IgM and IgG levels were low. Although clinical improvement followed transfer factor therapy, development of the malignant lymphoma was not prevented.

摘要

一名患有维斯科特-奥尔德里奇综合征的18岁男孩出现严重的血小板减少、反复感染和特应性湿疹症状。我们认为他是所描述的年龄第五大的维斯科特-奥尔德里奇综合征患者。最近,在他接受转移因子治疗期间,皮肤出现了组织细胞型恶性淋巴瘤。据我们所知,他是唯一报告的皮肤淋巴瘤患者,但另外四名维斯科特-奥尔德里奇综合征患者在转移因子治疗期间发生了恶性淋巴网状淋巴瘤。详细的免疫学研究表明,对各种抗原未能产生持续的抗体反应,缺乏迟发型超敏反应性,以及在体外培养中对抗原的增殖反应失败。IgE和IgA水平升高,IgM和IgG水平降低。尽管转移因子治疗后临床症状有所改善,但未能预防恶性淋巴瘤的发生。

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