title: Influence of thalassemia on the response to recombinant human erythropoietin in dialysis patients.
abstract: Thalassemia is a common genetic disorder among the South Chinese. To see if thalassemia would adversely affect the erythrocyte response to recombinant human erythropoietin (rHuEPO, Epogen) in dialysis patients, the response to rHuEPO in 4 dialysis patients with thalassemic traits (thal-t) was compared with that of 4 control patients who were matched for age, sex, mode of dialysis and baseline hemoglobin levels over a 6-month period. Patients with thal-t showed a reduced erythrocyte response to rHuEPO compared to control dialysis patients as reflected by a reduced reticulocyte index, a slower rise in hemoglobin or hematocrit levels, requirement of a higher cumulated dose of rHuEPO to achieve a target hemoglobin of 10 g/dl and a higher maintenance dose of rHuEPO. A dialysis patient with hemoglobin H disease (HbHD) was also studied. He failed to respond to rHuEPO despite that the dose was increased to 250 U/kg/week. In contrast, his matched control dialysis patient, despite a lower baseline hemoglobin level (6.1 versus 8.8 g/dl), was able to reach a target hemoglobin level of 10 g/dl by 6 weeks and could be maintained at this level with 50 U/kg/week. The patient with HbHD had splenomegaly and a higher baseline serum erythropoietin level, reticulocyte count, serum bilirubin, serum ferritin and serum iron saturation than control patients and patients with thal-t. It was concluded that thal-t reduces the erythrocyte response to rHuEPO in dialysis patients and that in the presence of active hemolysis and enhanced endogenous erythropoietin secretion, dialysis patients with HbHD are resistant to treatment with rHuEPO.
authors.affiliation: Department of Medicine, University of Hong Kong.
authors.forename: I K
authors.initials: IK
authors.lastname: Cheng
authors.forename: H B
authors.initials: HB
authors.lastname: Lu
authors.forename: D C
authors.initials: DC
authors.lastname: Wei
authors.forename: S W
authors.initials: SW
authors.lastname: Cheng
authors.forename: C Y
authors.initials: CY
authors.lastname: Chan
authors.forename: F C
authors.initials: FC
authors.lastname: Lee
mesh_terms: D000328:Adult; D000740:Anemia / Q000188:drug therapy* / Q000209:etiology; D004921:Erythropoietin / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications / Q000628:therapy*; D008297:Male; D011994:Recombinant Proteins / Q000627:therapeutic use; D006435:Renal Dialysis; D017085:alpha-Thalassemia / Q000150:complications*; D017086:beta-Thalassemia / Q000150:complications*
chemical_list: D011994:Recombinant Proteins; D004921:Erythropoietin