ATAXIA-TELANGIECTASIA WITH A 32 YEAR SURVIVAL - CLINICO-PATHOLOGICAL REPORT
ATAXIA-TELANGIECTASIA WITH A 32 YEAR SURVIVAL - CLINICO-PATHOLOGICAL REPORT
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DOI：
10.1097/00005072-197911000-00007
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发表时间：
1979-01-01
影响因子：
3.2
通讯作者：
TEPLITZ, R
中科院分区：
文献类型：
--
作者：
AMROMIN, GD;BODER, E;TEPLITZ, R
本文报告一位32岁患有共济失调-毛细血管扩张症的女性的临床病理结果。这是最古老的病人与这种疾病进行彻底研究，无论是临床和尸检。在脑和脊髓中发现了多发性小胶质血管畸形和肝脏毛细血管扩张。图示了其他共济失调毛细血管扩张的晚期病变。中枢神经系统和肝脏的血管畸形是独特的。患者死于恶性淋巴组织增生性疾病，并有5个其他恶性和良性肿瘤。
The clinico-pathological findings in a 32 yr old woman with ataxia-telangiectasia are presented. This is the oldest patient with this disease to be thoroughly studied both clinically and at autopsy. Multiple small gliovascular malformations in the brain and spinal cord and telangiectasis of the liver were found. Other advanced lesions of ataxia-telangiectasia are illustrated. The vascular malformations of the CNS and liver are unique. The patient died of a malignant lymphoproliferative disorder and had 5 other malignant and benign neoplasms.