Schilirò G, Comisi F F, Testa R, Dibenedetto S P, Samperi P, Marino S
Dipartimento di Pediatria, Università, Catania, Italy.
Haematologica. 1990 Mar-Apr;75(2):113-6.
We evaluated hematological parameters in 375 Sicilian adults with Hb S trait: Hb S levels were 41.91 +/- 2.65% in males and 40.92 +/- 2.8% in females. RBC, MCV, PCV, MCH, MCHC and total hemoglobin levels were within the normal range. Only mean Hb A2 and Hb F levels were increased (Hb A2 = 2.78 +/- 0.2%; Hb F = 1.05 +/- 0.18%), although they remained inside the normal ranges when compared to healthy controls (Hb A2 = 2.48 +/- 0.19%; Hb F = 0.93 +/- 0.14%) (p less than 0.0005). We conclude that our population does not show the hematological abnormalities such as microcytosis and decreased Hb levels, observed in the Black, Indian, Saudi Arabian carriers, and that the presence of those abnormalities is probably related to the coexistence of alpha-thalassemia, rarely observed in Sicily.
我们评估了375名携带Hb S性状的西西里岛成年人的血液学参数:男性的Hb S水平为41.91±2.65%,女性为40.92±2.8%。红细胞、平均红细胞体积、红细胞压积、平均红细胞血红蛋白含量、平均红细胞血红蛋白浓度和总血红蛋白水平均在正常范围内。只有平均Hb A2和Hb F水平升高(Hb A2 = 2.78±0.2%;Hb F = 1.05±0.18%),尽管与健康对照组相比(Hb A2 = 2.48±0.19%;Hb F = 0.93±0.14%)仍在正常范围内(p<0.0005)。我们得出结论,我们的研究人群未表现出在黑人、印度人、沙特阿拉伯携带者中观察到的诸如小红细胞症和血红蛋白水平降低等血液学异常,并且这些异常的存在可能与α地中海贫血的共存有关,而α地中海贫血在西西里岛很少见。