Koshy M, Entsuah R, Koranda A, Kraus A P, Johnson R, Bellvue R, Flournoy-Gill Z, Levy P
Department of Medicine, University of Illinois, Chicago 60612.
Blood. 1989 Sep;74(4):1403-8.
During the entry examination, leg ulcers were present in 2.5% of 2,075 patients 10 years of age and older with sickle cell disease who entered into the Cooperative Study of Sickle Cell Disease (CSSCD) between 1979 and 1986. Prevalence rates were highest among patients with sickle cell anemia and sickle cell anemia with thalassemia genotypes. Among sickle cell anemia patients free of ulcers at entry, the overall incidence was 5.73 per 100 person years in those having associated alpha-thalassemia and 9.97 for those without. Among sickle cell anemia patients with two alpha genes, the estimated incidence of leg ulcers is 2.38 per 100 person years and 6.12 per 100 person years among sickle cell anemia patients with three alpha genes (P less than .05). In both groups, the incidence was highest among those patients over 20 years of age and considerably higher among males than females (P less than .001). Leg ulcers were nonexistent in patients with sickle beta plus thalassemia and sickle hemoglobin C disease. Low steady-state hemoglobin is associated with a higher incidence of ulcer formation (P less than .0001) in sickle cell anemia patients. The protective effect of hemoglobin F is apparent at all levels of total hemoglobin among sickle cell anemia patients and those with associated alpha-thalassemia.
在1979年至1986年参加镰状细胞病合作研究（CSSCD）的2075名10岁及以上镰状细胞病患者的入院检查中，2.5%的患者存在腿部溃疡。患病率在镰状细胞贫血和镰状细胞贫血合并地中海贫血基因型的患者中最高。在入院时无溃疡的镰状细胞贫血患者中，伴有α地中海贫血的患者总体发病率为每100人年5.73例，无α地中海贫血的患者为每100人年9.97例。在具有两个α基因的镰状细胞贫血患者中，腿部溃疡的估计发病率为每100人年2.38例，在具有三个α基因的镰状细胞贫血患者中为每100人年6.12例（P小于0.05）。在这两组中，发病率在20岁以上的患者中最高，男性明显高于女性（P小于0.001）。镰状β加地中海贫血和镰状血红蛋白C病患者不存在腿部溃疡。低稳态血红蛋白与镰状细胞贫血患者溃疡形成的较高发病率相关（P小于0.0001）。在镰状细胞贫血患者和伴有α地中海贫血的患者中，血红蛋白F的保护作用在总血红蛋白的所有水平上都很明显。
