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共济失调毛细血管扩张症合并B细胞淋巴瘤:按照急性淋巴细胞白血病标准风险方案给予半量药物的效果

Ataxia telangiectasia associated with B-cell lymphoma: the effect of a half-dose of the drugs administered according to the acute lymphoblastic leukemia standard risk protocol.

作者信息

Yamada Y, Inoue R, Fukao T, Kaneko H, Isogai K, Fukuda S, Shimozawa N, Suzuki Y, Kondo N, Azuma E, Sakurai M

机构信息

Department of Pediatrics, Gifu University, School of Medicine, Japan.

出版信息

Pediatr Hematol Oncol. 1998 Sep-Oct;15(5):425-9. doi: 10.3109/08880019809016571.

DOI:10.3109/08880019809016571
PMID:9783309
Abstract

Ataxia telangiectasia (A-T) is a rare autosomal recessive disorder characterized by cerebellar ataxia, oculocutaneous telangiectasia, and variable degrees of humoral and cellular immunodeficiency. Affected individuals are known to exhibit a high incidence of lymphoma and leukemia. Because of increased chemosensitivity, the treatment of A-T patients with malignancies requires extremely careful planning and caution with respect to the use of chemotherapy. The authors report on a 12-year-old boy with A-T who developed B-cell lymphoma. He received a half-dose of the drugs administered according to the acute lymphoblastic leukemia (ALL) protocol issued by our children's cancer study group (9104 Standard Risk Protocol, Tokai Pediatric Oncology Study Group). As a result, he continues to be in complete remission and free of treatment complications 32 months after the diagnosis of B-cell lymphoma.

摘要

共济失调毛细血管扩张症(A-T)是一种罕见的常染色体隐性疾病,其特征为小脑共济失调、眼皮肤毛细血管扩张以及不同程度的体液免疫和细胞免疫缺陷。已知受影响个体患淋巴瘤和白血病的发生率很高。由于化疗敏感性增加,对患有恶性肿瘤的A-T患者进行治疗时,在使用化疗方面需要极其谨慎的规划和小心操作。作者报告了一名患有A-T的12岁男孩,他患上了B细胞淋巴瘤。他按照我们儿童癌症研究小组发布的急性淋巴细胞白血病(ALL)方案(9104标准风险方案,东海儿科肿瘤学研究小组)接受了半剂量的药物治疗。结果,在诊断出B细胞淋巴瘤32个月后,他持续处于完全缓解状态且没有治疗并发症。

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