Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin β-thalassemia intermedia patients

摘要: Clinical data suggest that in 8-thalassemia-intermedia patients, higher levels of circulating fetal hemoglobin (HbF) are associated with greater disease severity at comparable degrees anemia. We assessed the influence amount HbF on serum erythropoietin (S-Epo) and transferrin receptor, a measure erythropoiesis, 30 8-thalasemia-intermedia patients. Twenty-four showed more than 40% (21 whom B-thalassemia) 6 presented lower (p+ -thalassemia). The two groups patients did not differ age (1 5.3 v 19 years, respectively) or degree anemia (Hb = 8.8 g/dL both groups). Log was correlated inversely Hb (r - 0.47; P < .Ol), directly .55; .OOl). Multivariate regression analysis were independently ETA-THALASSEMIA intermedia is genetically heterogeneous disorder defined according to allows survive without blood transfusions grow severe bone deformities impairment sexual development.’,* Nevertheless, phenotype this varies respect first symptoms, splenomegaly, jaundice, cardiomegaly, incidence extramedullary erythropoiesis.“’ expansion resulting from an drive secondary chronic anemia, recognized as most important determinant phenotypic expression disease; however, it has also been claimed increased amounts may exert independent effect erythroid expansion.’-5 HbF, fact, shifts oxygen dissociation curve towards affinit~,~.~ thus potentially producing functional adjunctive stimulus erythropoiesis. Even though high have reported earlier presentation3 incidences erythropoiesis4 splenomegaly3 those low regulation erythropoiesis @-thalassemia never quantitatively investigated. This overwhelming importance view recent introduction therapies aimed increasing production ameliorate symptoms &globin In study we p-thalassemia intermedia. evaluated relation different concentrations. Moreover, because measurement transfemn receptor (S-TfR) recently proposed convenient method for monitoring erythropoiesis,” determined S-TfR them s-Epo. results investigation show that, &thalassemia values responsible S-Epo activity expected produces oversetting be clinically significant.