Theise N D, Ursell P C
Department of Pathology, Columbia University College of Physicians and Surgeons, New York, New York.
Am J Pediatr Hematol Oncol. 1990 Spring;12(1):74-6. doi: 10.1097/00043426-199021000-00014.
A 17-year-old boy with Gaucher's disease died suddenly 2 days after femoral osteotomy. At autopsy, in addition to extensive infiltrates of Gaucher cells in the cirrhotic liver, lymph nodes, and bone marrow, there were high-grade pulmonary arterial hypertensive changes but virtually no Gaucher cells in the lung. Although previous reports have stressed lung infiltrates with capillary plugging by Gaucher cells as important in the pathogenesis of pulmonary hypertension, the present case raises the question of a different mechanism--possibly a circulating vasoactive substance that bypasses the diseased liver.
一名患有戈谢病的17岁男孩在股骨截骨术后2天突然死亡。尸检时，除了在肝硬化肝脏、淋巴结和骨髓中有大量戈谢细胞浸润外，还存在重度肺动脉高压改变，但肺内几乎没有戈谢细胞。尽管先前的报告强调戈谢细胞导致的肺浸润及毛细血管堵塞在肺动脉高压发病机制中很重要，但本病例提出了一个不同机制的问题——可能是一种绕过患病肝脏的循环血管活性物质。
