Saraya A K, Kumar R, Choudhry V P, Kailash S, Sehgal A K
Am J Clin Pathol. 1985 Jul;84(1):103-7. doi: 10.1093/ajcp/84.1.103.
Serum ferritin, serum iron, and unsaturated iron binding capacity were studied in 64 patients with beta homozygous thalassemia (BHT), 120 patients with beta heterozygous thalassemia, and 46 normal subjects. Incidence of iron overload seen in 32 BHT cases was similar in untransfused and transfused cases. Among heterozygotes, iron stores were depleted in 24 (20%), mostly females (70.8%). Only male heterozygotes but not normals were iron deficient. In 18 (75%) heterozygotes with depleted iron stores, transferrin saturation (TS) was normal. It was also normal in 8 (25%) BHT patients and 5 (100%) heterozygotes with iron overload. In 13 (35.1%) BHT patients, it was raised in the absence of iron overload. It was concluded that iron deficiency in heterozygotes is of greater magnitude, especially in females, than hitherto known in India. Transferrin saturation is not a good indicator of either iron depletion or overload. Iron supplementation is recommended in heterozygous beta thalassemia in infants, children, and expectant mothers in geographic areas with high incidence of iron deficiency.
对64例β纯合子地中海贫血(BHT)患者、120例β杂合子地中海贫血患者和46名正常受试者的血清铁蛋白、血清铁和不饱和铁结合能力进行了研究。在32例BHT病例中,未输血和输血病例的铁过载发生率相似。在杂合子中,24例(20%)铁储存减少,其中大多数为女性(70.8%)。只有男性杂合子而非正常人缺铁。在18例(75%)铁储存减少的杂合子中,转铁蛋白饱和度(TS)正常。在8例(25%)BHT患者和5例(100%)有铁过载的杂合子中,TS也正常。在13例(35.1%)BHT患者中,TS在无铁过载的情况下升高。研究得出结论,杂合子中的缺铁程度比印度迄今所知的更为严重,尤其是在女性中。转铁蛋白饱和度不是铁缺乏或过载的良好指标。对于缺铁发生率高的地区,建议对婴儿、儿童和孕妇中的杂合子β地中海贫血患者进行铁补充。