Aksoy M, Erdem S
Acta Haematol. 1979;61(5):295-7. doi: 10.1159/000207675.
The unstable hemoglobins Istanbul and Saint-Etienne have the same amino acid substitution (alpha 2 beta 2 92F8 His leads to Gln). Despite this, there are some clinical and hematological differences between the individual with Hb Istanbul and the one with Hb Saint-Etienne. These are: (1) the clinical course of the patient with Hb Istanbul before splenectomy was more severe when compared with that of the individual with Hb Saint-Etienne; (2) although Hb F was found within normal range in the individual with Hb Istanbul, a high level of Hb F (19%) was observed in the patient with Hb Saint-Etienne. The alkali-resistant Hb found in the former was of adult type and in the latter case it was of fetal type. The possible explanations for these differences are discussed.
不稳定血红蛋白伊斯坦布尔型和圣艾蒂安型具有相同的氨基酸替换（α2β2 92F8 组氨酸变为谷氨酰胺）。尽管如此，携带血红蛋白伊斯坦布尔型的个体与携带血红蛋白圣艾蒂安型的个体之间仍存在一些临床和血液学差异。这些差异如下：（1）与携带血红蛋白圣艾蒂安型的个体相比，脾切除术前携带血红蛋白伊斯坦布尔型的患者临床病程更严重；（2）虽然携带血红蛋白伊斯坦布尔型的个体中血红蛋白F在正常范围内，但携带血红蛋白圣艾蒂安型的患者中观察到高水平的血红蛋白F（19%）。前者中发现的耐碱血红蛋白为成人型，而后者为胎儿型。文中讨论了这些差异的可能解释。
