Johnson K, Stastny J F, Rucknagel D L
Department of Internal Medicine, University of Cincinnati School of Medicine, Ohio.
Am J Hematol. 1994 Aug;46(4):354-7. doi: 10.1002/ajh.2830460418.
A 25-year-old African-American man with sickle cell-beta(+)-thalassemia presented with acute asthma of 2 days' duration. The asthma was well controlled by 48 hr, and parenteral medications were changed to oral bronchodilators and steroids. Sixty hours after presentation, he developed pain similar to that of sickle cell vaso-occlusion, for which he received small amounts of analgesics. He died approximately 84 hours after presentation. Autopsy showed extensive marrow necrosis and massive fat embolism. This is the first reported case of fat embolism syndrome associated with this genotype, the mildest of the clinically significant sickle cell hemoglobinopathies. The relationship between these etiologic factors and indications for transfusions are discussed.
一名患有镰状细胞-β（+）-地中海贫血的25岁非裔美国男性出现了持续2天的急性哮喘。48小时内哮喘得到了良好控制，肠胃外用药改为口服支气管扩张剂和类固醇。就诊60小时后，他出现了类似于镰状细胞血管阻塞的疼痛，为此他接受了少量镇痛药治疗。他在就诊后约84小时死亡。尸检显示广泛的骨髓坏死和大量脂肪栓塞。这是首例报道的与这种基因型相关的脂肪栓塞综合征病例，这种基因型是临床上有意义的镰状细胞血红蛋白病中最轻微的一种。本文讨论了这些病因与输血指征之间的关系。
