Intracellular hemoglobin S polymerization and the clinical severity of sickle cell anemia

Blood

Volumn 91, Issue 5, 1998, Pages 1777-1783

  Poillon, William N ^a   Kim, Bak C ^a   Castro, Oswaldo ^a,b  

^a HOWARD UNIVERSITY COLLEGE OF MEDICINE   (United States)

^b HOWARD UNIVERSITY   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

HEMOGLOBIN S;

ARTICLE; CELL POPULATION; DISEASE SEVERITY; HEMATOCRIT; HEMOLYTIC ANEMIA; HUMAN; HUMAN CELL; MEAN CORPUSCULAR HEMOGLOBIN; MICROCIRCULATION; OXYGEN SATURATION; POLYMERIZATION; PRIORITY JOURNAL; SICKLE CELL ANEMIA; SOLUBILITY;

EID: 0032030655     PISSN: 00064971     EISSN: None     Source Type: Journal    
DOI: 10.1182/blood.v91.5.1777.1777_1777_1783     Document Type: Article

References (47)

1. Poillon WN, Kim BC: 2,3-Diphosphoglycerate and intracellular pH as interdependent determinants of the physiologic solubility of deoxyhemoglobin S. Blood 76:1028, 1990
2. 2 at physiologic ligand saturation. Proc Natl Acad Sci USA 90:5039, 1993
3. Poillon WN, Kim BC, Labotka RH, Hicks CU, Kark JA: Antisickling effects of erythrocyte 2,3-diphosphoglycerate depletion. Blood 85:3289, 1995
4. Sunshine HR, Hofrichter J, Eaton WA: Requirements for therapeutic inhibition of sickle cell gelation. Nature 275:238, 1978
5. Eaton WA, Hofrichtrer J: Sickle cell hemoglobin gelation and sickle cell disease. Blood 70:1245, 1987
6. Eaton WA, Hofrichter J: Hemoglobin S polymerization. Adv Protein Chem 40:63, 1990
7. Serjeant GR, Serjeant BE, Desai P, Mason KP, Sewell A, England JM: The determinants of irreversibly sickled cells in homozygous sickle cell disease. Br J Haematol 40:431, 1978
8. 0-thalassemia with homozygous sickle cell disease. Br J Haematol 41:83, 1979
9. Powars DR, Schroeder WA, Weiss JN, Chan LS, Azen SP: Lack of influence of fetal hemoglobin levels or erythrocyte indices on the severity of sickle cell anemia. J Clin Invest 65:732, 1980
10. Embury SH, Dozy AM, Miller J, Davis JR, Kleman RM, Preesler H, Vishinsky E, Lande WN, Lubin B, Kan YW, Mentzer WC: Concurrent sickle cell anemia and α-thalassemia. N Engl J Med 306:270, 1982
11. Higgs DR, Aldridge BE, Lamb J, Clegg JB, Weatherall DJ, Hayes RH, Grandison Y, Lowrie Y, Mason KP, Serjeant BE, Serjeant GR: The interaction of α-thalassemia and homozygous sickle cell disease. N Engl J Med 306:1441, 1982
12. Steinberg MH, Dreiling BJ, Morrison FS, Necheles TF: Mild sickle cell disease. Clinical and laboratory studies. JAMA 224:317, 1973
13. Steinberg MH, Breibrig BJ, Lovell WJ: Sickle cell anemia: Erythrokinetics, blood volumes, and a study of possible determinants of severity. Am J Hematol 2:17, 1977
14. Steinberg MH, Rosenstock W, Coleman MB, Adams JG, Platca O, Cedeno M, Rieder RF, Wilson JT, Milner PF, West S: Cooperative study of sickle cell disease. Effects of thalassemia and microcytosis on the hemolytic and vasooclusive severity of sickle cell anemia. Blood 63:1353, 1984
15. Hayes RH, Beckford M, Grandison Y, Mason K, Serjeant BE, Serjeant GR: The haematology of steady state homozygous sickle cell disease: Frequency distributions, variation with age and sex, and longitudinal observations. Br J Haematol 59:369, 1985
16. Larner J, Smith ED, Surrey S, Schwartz E, Rappaport EF: Rheological properties of sickle erythrocytes in steady state predict frequency and severity of sickle cell anemia painful crisis. Blood 70:58a, 1987 (suppl 1)
17. Billett HH, Kim K, Fabry ME, Nagel RL: The percentage of dense red cells does not predict incidence of sickle cell painful crisis. Blood 68:301, 1986
18. Baum KF, Dunn DT, Maude GH, Serjeant GR: The painful crisis of homozygous sickle cell disease. A study of risk factors. Arch Intern Med 147:1231, 1987
19. Powars DR, Chan LS: Is sickle cell crisis a valid measure of clinical severity in sickle cell anemia? Prog Clin Biol Res 240:393, 1987
20. Hebbel RP, Boogaerts MAB, Eaton J, Steinberg MH: Erythrocyte adherence to endothelium in sickle cell anemia. A possible determinant of disease severity. N Engl J Med 302:992, 1980
21. Hebbel RP, Moldow CF, Steinberg MH: Modulation of erythrocytic-endothelial interactions and the vasooclusive severity of sickling disorders. Blood 58:947, 1981
22. Hebbel RP, Yamoda O, Moldow CF, Jacob HS, White JG, Eaton JW: Abnormal adherence of sickle erythrocytes to cultured vascular endothelium: Possible mechanism for microvascular occlusion in sickle cell disease. J Clin Invest 65:154, 1980
23. Eaton WA, Hofrichter J, Ross PD: Delay time of gelation: A possible determinant of clinical severity in sickle disease. Blood 47:621, 1976
24. Sunshine HR, Hofrichter J, Eaton WA: Gelation of sickle cell hemoglobin in mixtures with normal adult and fetal hemoglobins. J Mol Biol 133:435, 1979
25. Sunshine HR, Hofrichter J, Eaton WA: Oxygen binding by sickle cell hemoglobin polymers. J Mol Biol 158:251, 1982
26. Noguchi CT, Schechter AN: Intracellular polymerization of sickle hemoglobin and its relevance to sickle cell disease. Blood 58:1057, 1981
27. Noguchi CT, Schechter AN: Non-uniformity of intracellular polymer formation in sickle erythrocytes: Possible correlation with severity of hemolytic anemia. Am J Pediatr Hematol Oncol 6:46, 1984
28. Noguchi CT, Torchia DA, Schechter AN: Intracellular polymerization of sickle hemoglobin. Effects of cell heterogeneity. J Clin Invest 72:846, 1983
29. Noguchi CT, Rodgers GNP, Schechter AN: Intracellular polymerization of Hb S: Disease severity and therapeutic goals. Prog Clin Biol Res 240:381, 1987
30. Noguchi CT: Polymerization of erythrocytes containing S and non-S hemoglobins. Biophys J 45:1153, 1984
31. Noguchi CT, Rodgers GNP, Schechter AN : Intracellular polymerization, disease severity, and therapeutic predictions. N Y Acad Sci 565:75, 1989
32. Ross PD, Hofrichter J, Eaton WA: Thermodynamics of gelation of sickle cell deoxyhemoglobin. J Mol Biol 112:111, 1977
33. Ross PD, Minton AP: Analysis of nonideal behavior in concentrated hemoglobin solutions J Mol Biol 112:437, 1977
34. Brittenham GM, Schechter AN, Noguchi CT: Hemoglobin S polymerization: Primary determinant of the hemolytic and clinical severity of the sickling syndromes. Blood 65:183, 1985
35. Keidan AJ, Sowter MC, Johnson CS, Noguchi CT, Girling AJ, Stevens ME, Stuart J: Effect of polymerization tendency on hematological, rheological and clinical parameters in sickle cell anemia. Br J Haematol 71:551, 1989
36. van Assendelft OW: Spectrophotometry of Hemoglobin Derivatives. Springfield, IL, Thomas, 1970
37. Singer K, Chernoff AI, Singer L: Studies on abnormal hemoglobins. I. Their demonstration in sickle cell anemia and other hematologic disorders by means of alkali denaturation. Blood 6:413, 1951
38. 2. J Lab Clin Med 86:700, 1975
39. Goldberg MA, Husson MA, Bunn HF: The participation of hemoglobins A and F in the polymerization of sickle hemoglobin. J Biol Chem 252:3414, 1977
40. Ueda Y, Nagel RL, Bookchin RM: An increased Bohr effect in sickle cell anemia. Blood 53:472, 1979
41. Steinberg MH, Hebbel RP: Clinical diversity of sickle cell anemia. Genetic and cellular modulation of disease severity. Am J Hematol 14:405, 1983
42. Platt O, Thorington BD, Brambilla DJ, Milner PF, Rosse WF, Vishinsky E, Kinney TR: Pain in sickle cell disease. Rates and risk factors. N Engl J Med 325:11, 1991
43. Coletta M, Hofrichter J, Ferrone FA, Eaton WA: Kinetics of sickle hemoglobin polymerization in single red cells. Nature 300:194, 1982
44. Mozzarelli A, Hofrichter J, Eaton WA: Delay time of hemoglobin S polymerization prevents most cells from sickling in vivo. Science 237:500, 1987
45. Hofrichter J, Ross PD, Eaton WA: Supersaturation in sickle cell hemoglobin solutions. Proc Natl Acad Sci USA 73:3035, 1976
46. Bertles JF, Milner PFA: Irreversibly sickled cells: A consequence of the heterogeneous distribution of hemoglobin types in sickle cell anemia. J Clin Invest 47:1731, 1968
47. Seakins M, Gibbs WN, Milner PF, Bertles JF: Erythrocyte Hb-S concentration. An important factor in the low oxygen affinity of blood in sickle cell anemia. J Clin Chem 52:422, 1973