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骨石化症中出现的胸内髓外造血。阐明遗传疾病机制。

Intrathoracic extramedullary hematopoiesis arising in marble bone disease. Clarifying the genetic disease mechanism.

作者信息

Shigemura Norihisa, Akashi Akinori, Nakagiri Tomoyuki

机构信息

Division of the General Thoracic Surgery, Takarazuka Municipal Hospital, 4-5-1 Kohama, Takarazuka 665-0827, Japan.

出版信息

Jpn J Thorac Cardiovasc Surg. 2002 Jul;50(7):311-4. doi: 10.1007/BF03032303.

Abstract

We report a man with intrathoracic extramedullary hematopoiesis accompanied by marble bone disease who underwent thoracoscopic resection safely and definitively, followed by a radionuclide scan. Although intrathoracic extramedullary hematopoiesis is almost always accompanied by hemolytic anemia, this man's hematological data normalized. A consensus on the tumor genesis mechanism has yet to be reached, but the existence of marble bone disease in our case throws new light on the genetic mechanism in intrathoracic extramedullary hematopoiesis.

摘要

我们报告了一名患有胸腔内髓外造血并伴有大理石骨病的男性患者,该患者安全且彻底地接受了胸腔镜切除术,随后进行了放射性核素扫描。尽管胸腔内髓外造血几乎总是伴有溶血性贫血,但该男子的血液学数据恢复了正常。关于肿瘤发生机制尚未达成共识,但我们病例中存在大理石骨病为胸腔内髓外造血的遗传机制提供了新的线索。

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