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血红蛋白H病(HbH)是α地中海贫血中最常见的类型。作者在1981—83年间发现4例。其中男1例,女3例。年龄17—43岁。均有贫血史。3例女性病人因进入青春期后症状明显;1例男性病人,肝脾肿大15年,因感染发热,贫血加重而引起重视,初诊至确诊时间均超过4年。体症:2例有巩膜黄染,1例枕部有臀状沟样颅骨畸形。4例Hb4.5—9.3g%,平均7g%,均有肝脾肿大,网织红0.7—8.1%,红细胞脆性试验完全溶血均<0.30%,长期误诊为缺铁性贫血、慢性肝炎。
Hemoglobin H disease (HbH) is the most common type of alpha thalassemia. The authors found 4 cases in 1981-83. There were 1 males and 3 females. Age 17-43 years old. Have a history of anemia. 3 cases of female patients had obvious symptoms after entering adolescence; 1 case of male patients with hepatosplenomegaly enlarged 15 years due to infection fever and anemia, and the time from diagnosis to diagnosis was more than 4 years. Symptoms: 2 cases of scleral yellow dye, 1 case of hip croup-like skull deformity. 4 cases of Hb4.5-9.3g%, an average of 7g%, both hepatosplenomegaly, reticulocyte 0.7-8.1%, complete hemolysis of erythrocyte fragility test <0.30%, long-term misdiagnosed as iron deficiency anemia, chronic hepatitis.