另一个患有北岸血红蛋白病（β134缬氨酸→谷氨酸）的澳大利亚家庭。

A second Australian family with hemoglobin North Shore (beta 134 Val----Glu).

作者信息

Gurney H, Baig I, Gordon S, Phadke K, Kearsley H, Fleming P, Wyatt K, Hughes W

出版信息

Pathology. 1987 Jan;19(1):62-3. doi: 10.3109/00313028709065138.

Abstract

A second Australian family is reported with Hemoglobin North Shore (beta 134 Val----Glu), an unstable hemoglobin, causing no clinical symptoms. All affected family members showed only mild reticulocytosis and microcytosis on the blood film, despite the strongly positive isopropanol test for Hb (hemoglobin) stability and numerous red cell inclusions. Hb North Shore constituted 31-38% of the total hemoglobin and migrated on the anodal side of Hb A at pH 8.9. The association of a mildly raised Hb A2 level and thalassemic phenotype with Hb North Shore previously reported, is confirmed in this study.

摘要

据报道，有第二个澳大利亚家庭患有北岸血红蛋白（β134缬氨酸→谷氨酸），这是一种不稳定的血红蛋白，但未引起临床症状。尽管针对血红蛋白（Hb）稳定性的异丙醇试验呈强阳性且有大量红细胞包涵体，但所有受影响的家庭成员在血涂片上仅表现出轻度的网织红细胞增多和小红细胞症。北岸血红蛋白占总血红蛋白的31% - 38%，在pH 8.9时迁移至Hb A的阳极侧。本研究证实了先前报道的北岸血红蛋白与轻度升高的Hb A2水平和地中海贫血表型之间的关联。