Characterization of haemolyser‐resistant cells increased in the blood of erythropoietin‐treated mice

Said, Ahmed A.; Yamaguchi, Teruhide; Uchida, Eiji; Hayakawa, Tetsuo

doi:10.1111/j.1365-2141.1994.tb04740.x

Cited by 4 publications

(5 citation statements)

References 19 publications

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“…The rationale for the treatment with rHuEPO in β-thalassemia originates from studies in baboons and mice [7, 8, 9]. Results demonstrated the benefit of rHuEPO on the stimulation of HbF synthesis.…”

Section: Discussionmentioning

confidence: 99%

“…Pharmacological stimulation of fetal hemoglobin (HbF) synthesis by agents such as cytarabine, Aza-C, butyrate, hydroxyurea and recombinant human erythropoietin (rHuEPO) has attracted a lot of interest [5, 6, 7, 8, 9, 10]. EPO promotes the differentiation, proliferation and survival of erythroid precursors and RBCs, induces HbF synthesis and could be useful in the treatment of some selected transfusion-dependent patients with β-thalassemia major and intermedia [11, 12, 13, 14].…”

Section: Introductionmentioning

confidence: 99%

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Treatment of β-Thalassemia Patients with Recombinant Human Erythropoietin: Effect on Transfusion Requirements and Soluble Adhesion Molecules

et al. 2004

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The most common single genetic disorder and a major public health issue in Greece and other Mediterranean countries is β-thalassemia. Current therapeutic approaches for homozygous β-thalassemia entail blood transfusions and iron chelation therapy with deferoxamine or deferiprone for preventing tissue hemosiderosis. Recently, much effort has focused on various inducers of fetal hemoglobin (HbF) such as recombinant human erythropoietin (rHuEPO), especially in β-thalassemia intermedia. Ten adult patients, 5 with β-thalassemia major and 5 with β-thalassemia intermedia, received 150 IU/kg rHuEPO (epoetin-α) subcutaneously three times a week. Seven patients were transfused every 14–30 days and 3 with β-thalassemia intermedia were only occasionally transfused. The minimum duration of treatment was 12 weeks in order to define if there was any response. Transfusion intervals were modified according to the rHuEPO response to maintain stable Hb values. Lower transfusion requirements were observed in 5 patients after rHuEPO treatment (p = 0.028). In the 3 non-transfused patients, Hb values increased, and the patients are still being treated and followed up for a period ranging from 14 weeks to 2 years. Two patients with thalassemia major discontinued treatment after 12 weeks, as they did not achieve any response regarding transfusion requirements or Hb values. Pretreatment serum transferrin receptor levels were higher than in controls (p < 0.001) and significantly increased following rHuEPO treatment (p = 0.027). Patients had higher serum endothelin-3, sICAM-1 and sE-selectin values before rHuEPO treatment compared to controls (p < 0.001, p < 0.001 and p = 0.016, respectively), but these values were not altered during treatment. HbF values presented a slight, non-significant increase. rHuEPO treatment has a beneficial effect in transfusion-dependent β-thalassemia patients. Although a slight increase in HbF levels was observed, other possible mechanisms are probably involved. None of our patients experienced thrombotic complications and a rise in blood pressure.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Treatment of β-Thalassemia Patients with Recombinant Human Erythropoietin: Effect on Transfusion Requirements and Soluble Adhesion Molecules

et al. 2004

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“…Endogenous serum Epo levels in various forms of β-thalassemia have been found inconsistent and not related to the degree of anemia [17]. The rationale for the treatment with rHuEpo in β-thalassemia came from studies in baboons [6] and mice [7,8]. The results demonstrated the positive effect of rHuEpo on the stimulation of HbF synthesis.…”

Section: Discussionmentioning

confidence: 99%

“…The development of somatic gene therapy to replace the defective globin genes remains a goal for the future despite intensive investigation in this area [3]. Much effort has focused on pharmacological stimulation of fetal hemoglobin synthesis by agents such as cytarabine, butyrate, hydroxyurea, and recombinant human erythropoietin (rHuEpo) [4,5,6,7,8,9]. rHuEpo promotes the differentiation and proliferation of erythroid cells, induces the production of fetal hemoglobin (HbF), and could be useful in the treatment of some selected transfusion-dependent patients with homozygous β-thalassemia intermedia and β-thalassemia major [10, 11.12.13, 14].…”

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confidence: 99%

Recombinant human erythropoietin therapy in a transfusion-dependent β-thalassemia major patient

Chaliasos

Hatzimichael

et al. 2001

Annals of Hematology

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We report on a 28-year-old patient with transfusion-dependent beta-thalassemia major, who was treated effectively with recombinant human erythropoietin (rHuEpo). rHuEpo promotes the differentiation and proliferation of erythroid cells, induces the production of fetal hemoglobin (HbF), and could be useful in the treatment of some selected transfusion-dependent thalassemia patients. Prior to rHuEpo treatment, the patient was on a regular blood transfusion regimen. Splenectomy did not decrease the transfusion requirements. Additionally, red cell alloimmunization had developed; therefore, we decided to start rHuEpo treatment (Eprex, Jansen Cilag, Greece) in an attempt to improve his anemia and the quality of life. Our patient responded well to rHuEpo treatment and was able to extend the intervals between transfusions from 10-14 to 55-65 days and to sustain a pretransfusion hemoglobin level above 7 g/dl. HbF levels were slightly increased from 55% to 60-65%. Indicators of vascular endothelial activation [serum endothelin-3, intracellular adhesion molecule-1 (ICAM-1), vascular cell adhesion molecule-1 (VCAM-1), and E-selectin] were decreased during treatment. rHuEpo was well tolerated without complications. rHuEpo treatment seemed to have had a beneficial effect and to have improved the quality of life in beta-thalassemia major, although it did have a slight effect on HbF levels, suggesting other possible mechanisms of rHuEpo action.

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“…Bone marrow transplanta tion may be curative but is not feasible in every case since an HLA-matched sibling is needed. Gene therapy is also not available at present [4][5][6], Augmentation of y-gene synthesis by pharmacological agents including cytarabine, arginine butyrate, hydroxy urea and recombinant human erythropoietin (r-Hu-EPO) represents a new approach to the therapy of p-thalassemia [7][8][9][10][11][12].…”

Section: Introductionmentioning

confidence: 99%

Recombinant Erythropoietin Trial in Children with Transfusion-Dependent Homozygous β-Thalassemia

Nişli

Kavaklı²,

Aydınok³

et al. 1997

Acta Haematol

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Augmentation of γ-gene synthesis by using recombinant human erythropoietin (r-Hu-EPO) represents a new approach to the therapy of β-thalassemia. A prospective study was conducted in 26 transfusion-dependent β-thalassemia major patients. r-Hu-EPO (Eprex/Cilag, Switzerland) was given to the patients at an initial dose of 500 IU/kg s.c. 3 times a week for at least 2 months during which no transfusion was applied. A sustained hemoglobin (Hb) level greater than 8 g/dl was considered as a response to EPO treatment. In the patients whose Hb levels remained under 8 g/dl or did not increase in comparison to pretreatment levels within 4 weeks, the dose of r-Hu-EPO was increased to 1,000 IU/kg 3 times a week and applied for another 4 weeks. Only 16 cases also received oral iron supplementation. The whole blood and reticulocyte counts, the biochemical tests including BUN, creatinine, AST, ALT, alkaline phosphatase and ferritin were done and the percentages of HbF and F cells were analyzed regularly. At the end of the 2nd month, 6 cases qualified to continue with the trial. At the end of the 6th month, r-Hu-EPO therapy was ceased in 3 cases of the 6 since their Hb levels had decreased below 7 g/dl. Only 3 cases (11.5%) continued with the r-Hu-EPO therapy without transfusion for up to 12 months. In conclusion, r-Hu-EPO may be useful in some selected transfusion-dependent patients with β-thalassemia major. Selection criteria should include a mild β-genotype or coinheritance of α-thalassemia, splenectomy and pretreatment reticulocyte response of the patients as well as the patients’ compliance.

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Characterization of haemolyser‐resistant cells increased in the blood of erythropoietin‐treated mice

Cited by 4 publications

References 19 publications

Treatment of β-Thalassemia Patients with Recombinant Human Erythropoietin: Effect on Transfusion Requirements and Soluble Adhesion Molecules

Treatment of β-Thalassemia Patients with Recombinant Human Erythropoietin: Effect on Transfusion Requirements and Soluble Adhesion Molecules

Recombinant human erythropoietin therapy in a transfusion-dependent β-thalassemia major patient

Recombinant Erythropoietin Trial in Children with Transfusion-Dependent Homozygous β-Thalassemia

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