Cazzola M, De Stefano P, Ponchio L, Locatelli F, Beguin Y, Dessi C, Barella S, Cao A, Galanello R
Department of Internal Medicine and Medical Oncology, University of Pavia, Italy.
Br J Haematol. 1995 Mar;89(3):473-8. doi: 10.1111/j.1365-2141.1995.tb08351.x.
In the management of beta-thalassaemia major, different transfusion schemes are employed with baseline haemoglobin levels ranging from 8 to over 12 g/dl. We studied the relationship between transfusion regimen and suppression of erythropoiesis in 52 patients with beta-thalassaemia major whose mean pretransfusion haemoglobin levels ranged from 8.6 to 10.9 g/dl. Multiple, regression analysis showed that serum transferrin receptor was the parameter more closely related to mean pretransfusion haemoglobin (r = -0.77, P < 0.001). As measured through serum transferrin receptor, erythroid activity was 1-2 times normal for pretransfusion haemoglobin levels between 10 and 11 g/dl. 1-4 times normal for levels from 9 to 10 g/dl, and 2-6 times normal for levels from 8.6 to 9 g/dl. Mean pretransfusion haemoglobin was also inversely related to serum erythropoietin (r = -0.72, P < 0.001), whereas it showed no or a weak relationship with Hb F, reticulocyte count, or circulating nucleated red cell count. This study suggests that serum transferrin receptor is a reliable indicator of suppression of erythropoiesis in beta-thalassaemia major. On the basis of our findings, pretransfusion haemoglobin values of < or = 9 g/dl should be adopted with caution, because these levels can be associated with an insufficient inhibition of erythroid marrow expansion. However, a transfusion programme, with a baseline haemoglobin of 9-10 g/dl, may provide enough suppression of erythropoiesis and allow a reduction in blood consumption as compared with the classic hyper- or supertransfusion schemes. Since fixed haemoglobin levels may not be the best target for transfusion treatment in all thalassaemic patients, assay of serum transferrin receptor may be helpful for individualizing the transfusion regimens.
在重型β地中海贫血的治疗中，采用了不同的输血方案，基线血红蛋白水平范围为8至超过12 g/dl。我们研究了52例重型β地中海贫血患者的输血方案与红细胞生成抑制之间的关系，这些患者输血前血红蛋白水平的平均值范围为8.6至10.9 g/dl。多元回归分析表明，血清转铁蛋白受体是与输血前平均血红蛋白更密切相关的参数（r = -0.77，P < 0.001）。通过血清转铁蛋白受体测量，对于输血前血红蛋白水平在10至11 g/dl之间，红系活性是正常的1 - 2倍；对于9至10 g/dl的水平，是正常的1 - 4倍；对于8.6至9 g/dl的水平，是正常的2 - 6倍。输血前平均血红蛋白也与血清促红细胞生成素呈负相关（r = -0.72，P < 0.001），而它与Hb F、网织红细胞计数或循环有核红细胞计数无或呈弱相关。这项研究表明，血清转铁蛋白受体是重型β地中海贫血红细胞生成抑制的可靠指标。基于我们的研究结果，输血前血红蛋白值≤9 g/dl应谨慎采用，因为这些水平可能与红系骨髓扩张抑制不足有关。然而，与经典的强化或超输血方案相比，基线血红蛋白为9 - 10 g/dl的输血方案可能提供足够的红细胞生成抑制，并减少血液消耗。由于固定的血红蛋白水平可能并非所有地中海贫血患者输血治疗的最佳目标，血清转铁蛋白受体检测可能有助于个体化输血方案。
