CONCURRENT SICKLE-CELL-ANEMIA AND ALPHA-THALASSEMIA - EFFECT ON SEVERITY OF ANEMIA
CONCURRENT SICKLE-CELL-ANEMIA AND ALPHA-THALASSEMIA - EFFECT ON SEVERITY OF ANEMIA
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DOI：
10.1056/nejm198202043060504
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发表时间：
1982-01-01
影响因子：
158.5
通讯作者：
MENTZER, WC
中科院分区：
文献类型：
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作者：
EMBURY, SH;DOZY, AM;MENTZER, WC
研究患有镰状细胞性贫血的患者(47)以确定α-地中海贫血对其溶血性贫血严重程度的影响。通过使用α-珠蛋白基因图谱检测α-珠蛋白基因缺失来客观地诊断α-地中海贫血，研究了25名具有正常4个α-珠蛋白基因的受试者，其中18名受试者有3个，4名受试者有2个。平均Hb、血细胞比容和绝对网织红细胞水平(.+-.SD)为7.9.+-。 0.9 克/分升 (4.9.+-.0.6 毫摩尔/升), 22.9.+-. 2.9% 和 501,000 .+-。非地中海贫血组分别为 126,000/mm3； 9.8.+-. 1.6 克/分升（6.1.+-.1.0 毫摩尔/升），29.0.+-。 5.0% 和 361,000 .+-。具有3个α-珠蛋白基因的组为51,000/mm3；和 9.2.+-。 1.0 克/分升（5.7.+-.0.6 毫摩尔/升），27.5.+-。 3.0% 和 100,000 .+-。具有2个α-珠蛋白基因的组中为15,000/mm3。 α-珠蛋白基因的缺失还伴随网织红细胞后平均红细胞Hb浓度(MCHC)的降低和Hb F水平的增加。与α-地中海贫血相关的红细胞内Hb S浓度降低和Hb F水平升高似乎减轻了镰状细胞病中发现的溶血性贫血的程度。
Patients (47) with sickle-cell anemia were studied to determine the effect of .alpha.-thalassemia on the severity of their hemolytic anemia. .alpha.-Thalassemia was diagnosed objectively by using .alpha.-globin-gene mapping to detect .alpha.-globin-gene deletions, studying 25 subjects with the normal 4 .alpha.-globin genes, 18 with 3, and 4 with 2. The mean Hb, hematocrit and absolute reticulocyte levels (.+-. SD) were 7.9 .+-. 0.9 g/dl (4.9 .+-. 0.6 mmol/l), 22.9 .+-. 2.9%, and 501,000 .+-. 126,000/mm3, respectively, in the non-thalassemic group; 9.8 .+-. 1.6 g/dl (6.1 .+-. 1.0 mmol/l), 29.0 .+-. 5.0%, and 361,000 .+-. 51,000/mm3 in the group with 3 .alpha.-globin genes; and 9.2 .+-. 1.0 g/dl (5.7 .+-. 0.6 mmol/l), 27.5 .+-. 3.0%, and 100,000 .+-. 15,000/mm3 in the group with 2 .alpha.-globin genes. Deletion of .alpha.-globin genes was also accompanied by a decreased mean corpuscular Hb concentration (MCHC) in post-reticulocyte erythrocytes and by increased Hb F levels. The decreased intraerythrocytic Hb S concentration and elevated Hb F levels associated with .alpha.-thalassemia appear to diminish the degree of hemolytic anemia found in sickle-cell disease.