论文部分内容阅读
血红蛋白H病或中间型α地中海贫血是以出生后存在大量(5～30％)血红蛋白H(简称HbH)且脾切除患者的许多红细胞中有沉淀的HbH包涵体(本病患者不经脾切除其红细胞内亦多可检得HbH包涵体——编者)为特征的一种溶血性疾病。本文报告502例的研究结果。新生儿患者的临床表现 HbH病的新生儿,无异常表现,肝脾无肿大,血红蛋白水平接近正常,但可见地中海贫血的红细胞形态改变,约25％的患儿可测出HbBait’s。HbH仅微量存在,但随后HbH便逐渐代替了Hb Bart’s。HbH病的典型表现常在第一年出现。
Hemoglobin H disease or intermediate alpha thalassemia is a HbH inclusion body that is precipitated in many erythrocytes in patients who have a large amount (5 to 30%) of hemoglobin H (HbH) after spawning and are spleen-free Hepatic cells are also more seizure HbH inclusion body - editor) is characterized by a hemolytic disease. This article reports the results of 502 studies. Neonatal patients with HbH disease in newborns, no abnormalities, no enlarged liver and spleen, hemoglobin levels close to normal, but the red blood cells showed thalassemia changes in morphology, about 25% of children can be detected HbBait’s. HbH only exists in a trace amount, but then HbH gradually replaced Hb Bart’s. The typical manifestation of HbH disease often occurs in the first year.