Hydroxycarbamide-induced changes in E/beta thalassemia red blood cells

American Journal of Hematology

Volumn 83, Issue 11, 2008, Pages 842-845

  Singer, Sylvia T ^a,b   Vichinsky, Elliott P ^a   Larkin, Sandra ^d   Olivieri, Nancy ^c   Sweeters, Nancy ^b   Kuypers, Frans A ^d  

^a Clinical Research Center   (United States)

^b CHILDREN S HOSPITAL OAKLAND   (United States)

^c TORONTO GENERAL HOSPITAL   (Canada)

^d CHILDREN S HOSPITAL OAKLAND RESEARCH INSTITUTE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

HEMOGLOBIN F; HYDROXYUREA; MEMBRANE PHOSPHOLIPID; PHOSPHATIDYLSERINE;

ADOLESCENT; ADULT; ARTICLE; BETA THALASSEMIA; BONE MARROW TOXICITY; CELL HYDRATION; CHILD; CLINICAL ARTICLE; CONTROLLED STUDY; DRUG DOSE ESCALATION; ERYTHROCYTE DEFORMABILITY; ERYTHROCYTE LIFESPAN; ERYTHROCYTE MEMBRANE; ERYTHROCYTE PARAMETERS; FEMALE; HEMOGLOBIN BLOOD LEVEL; HUMAN; HUMAN CELL; MALE; MEMBRANE STRUCTURE; PHOSPHOLIPID MEMBRANE; PRIORITY JOURNAL; SPLENECTOMY; STATISTICAL SIGNIFICANCE; TREATMENT RESPONSE;

ADOLESCENT; ADULT; BETA-THALASSEMIA; CHILD; CHILD, PRESCHOOL; COHORT STUDIES; ERYTHROCYTE DEFORMABILITY; ERYTHROCYTE INDICES; FEMALE; FETAL HEMOGLOBIN; HEMOGLOBIN E; HUMANS; HYDROXYUREA; MALE; NUCLEIC ACID SYNTHESIS INHIBITORS; OSMOTIC FRAGILITY;

EID: 55549147561     PISSN: 03618609     EISSN: 10968652     Source Type: Journal    
DOI: 10.1002/ajh.21266     Document Type: Article

References (20)

1. Schrier SL, Mohandas N. Globin-chain specificity of oxidation-induced changes in red blood cell membrane properties. Blood 1992;79:1586-1592.
2. Advani R, Rubin E, Mohandas N, Schrier SL. Oxidative red blood cell membrane injury in the pathophysiology of severe mouse beta-thalassemia. Blood 1992;79:1064-1067.
3. Pootrakul P, Sirankapracha P, Hemsorach S, et al. A correlation of erythrokinetics, ineffective erythropoiesis, and erythroid precursor apoptosis in thai patients with thalassemia. Blood 2000;96:2606-2612.
4. Nienhuis AW, Ley TJ, Humphries RK, et al. Pharmacological manipulation of fetal hemoglobin synthesis in patients with severe beta-thalassemia. Ann N Y Acad Sci 1985;445:198-211.
5. Olivieri NF. Fetal erythropoiesis and the diagnosis and treatment of hemoglobin disorders in the fetus and child. Semin Perinatol 1997;21:63-69.
6. Fucharoen S, Siritanaratkul N, Winichagoon P, et al. Hydroxyurea increases hemoglobin F levels and improves the effectiveness of erythropoiesis in beta-thalassemia/hemoglobin E disease. Blood 1996;87:887-892.
7. Singer ST, Kuypers FA, Olivieri NF, et al. Fetal haemoglobin augmentation in E/beta(0) thalassaemia: Clinical and haematological outcome. Br J Haematol 2005;131:378-388.
8. Fathallah H, Sutton M, Atweh GF. Pharmacological induction of fetal hemoglobin: Why haven't we been more successful in thalassemia? Ann N Y Acad Sci 2005;1054:228-237.
9. Mohandas N, Clark MR, Jacobs MS, Shohet SB. Analysis of factors regulating erythrocyte deformability. J Clin Invest 1980;66:563-573.
10. Kiefer CR, Snyder LM. Oxidation and erythrocyte senescence. Curr Opin Hematol 2000;7:113-116.
11. Kuypers FA, Yuan J, Lewis RA, et al. Membrane phospholipid asymmetry in human thalassemia. Blood 1998;91:3044-3051.
12. Atichartakarn V, Angchaisuksiri P, Aryurachai K, et al. Relationship between hypercoagulable state and erythrocyte phosphatidylserine exposure in splenectomized haemoglobin E/beta-thalassaemic patients. Br J Haematol 2002;118:893-898.
13. Eldor A, Rachmilewitz EA. The hypercoagulable state in thalassemia. Blood 2002;99:36-43.
14. Angchaisuksiri P, Atichartakarn V, Aryurachai K, et al. Hemostatic and thrombotic markers in patients with hemoglobin E/beta-thalassemia disease. Am J Hematol 2007;82:1001-1004.
15. Watanapokasin R, Sanmund D, Winichagoon P, Muta K, Fucharoen S. Hydroxyurea responses and fetal hemoglobin induction in beta-thalassemia/HbE patients' peripheral blood erythroid cell culture. Ann Hematol 2006;85:164-169.
16. Amoyal I, Goldfarb A, Fibach E. Flow cytometric analysis of hydroxyurea effects on fetal hemoglobin production in cultures of beta-thalassemia erythroid precursors. Hemoglobin 2003;27:77-87.
17. Bradai M, Abad MT, Pissard S, et al. Hydroxyurea can eliminate transfusion requirements in children with severe beta-thalassemia. Blood 2003;102:1529-1530.
18. Orringer EP, Blythe DS, Johnson AE, et al. Effects of hydroxyurea on hemoglobin F and water content in the red blood cells of dogs and of patients with sickle cell anemia. Blood 1991;78:212-216.
19. Eastman JW, Wong R, Liao CL, Morales DR. Automated HPLC screening of newborns for sickle cell anemia and other hemoglobinopathies. Clin Chem 1996;42:704-710.
20. Kuypers FA, Scott MD, Schott MA, Lubin B, Chiu DT. Use of ektacytometry to determine red cell susceptibility to oxidative stress. J Lab Clin Med 1990;116:535-545.