伴有遗传性球形红细胞增多症和镰状细胞性状导致的脾梗死。

Splenic infarction due to concomitant hereditary spherocytosis and sickle cell trait.

作者信息

Dulman Robin Yates, Buchanan George R, Ginsburg Howard, Fefferman Nancy R, Greco M Alba, Borys Dariusz, Blei Francine

机构信息

Department of Pediatric Hematology/Oncology, NYU School of Medicine, New York, NY 10016, USA.

出版信息

J Pediatr Surg. 2007 Dec;42(12):2129-31. doi: 10.1016/j.jpedsurg.2007.07.057.

DOI:10.1016/j.jpedsurg.2007.07.057

PMID:18082724

Abstract

Concomitant hereditary spherocytosis and sickle cell trait, although extremely rare, could potentially lead to splenic sequestration or infarction. We report here the first case of splenic infarction in a child with hereditary spherocytosis and sickle cell trait while flying on a commercial aircraft. The presence of hypoxia, hemoconcentrated erythrocytes, and sickle hemoglobin created the perfect environment for clinical sequelae.

摘要

遗传性球形红细胞增多症与镰状细胞性状并存，虽然极为罕见，但可能会导致脾隔离症或梗死。我们在此报告首例患有遗传性球形红细胞增多症和镰状细胞性状的儿童在乘坐商业飞机飞行时发生脾梗死的病例。缺氧、血液浓缩的红细胞以及镰状血红蛋白的存在为临床后遗症创造了完美的环境。