血红蛋白镰状O-阿拉伯病中的急性脾滞留

Acute splenic sequestration in hemoglobin sickle O-Arab disease.

作者信息

Gilman P A, Abel A S

出版信息

Johns Hopkins Med J. 1980 Jun;146(6):285-8.

Abstract

A white girl with sickle O-Arab disease (Hb S/O-Arab) had three separate episodes of acute splenic sequestration at 8, 12, and 15 months of age with hemoglobin levels falling to 3.9, 2.8, and 4.2 g/dl, respectively. Functional hyposplenism was suggested by the radionucleotide spleen scan. Following splenectomy her hemoglobin stabilized in the range of 0.7--8.5 g/dl. Life-threatening episodes of splenic sequestration, pneumococcal infections and osteomyelitis may occur in both Hb S/O-Arab and homozygous sickle cell diseases.

摘要

一名患有镰状O-阿拉伯病（Hb S/O-Arab）的白人女孩在8个月、12个月和15个月大时分别经历了三次急性脾滞留发作，血红蛋白水平分别降至3.9、2.8和4.2 g/dl。放射性核素脾脏扫描提示功能性脾功能减退。脾切除术后，她的血红蛋白稳定在0.7-8.5 g/dl范围内。Hb S/O-Arab病和纯合子镰状细胞病都可能发生危及生命的脾滞留、肺炎球菌感染和骨髓炎发作。