Quantitative analysis of erythrocytes containing fetal hemoglobin (F cells) in children with sickle cell disease

American Journal of Hematology

Volumn 54, Issue 1, 1997, Pages 40-46

  Marcus, Stacy J ^a   Kinney, Thomas R ^a   Schultz, William H ^a   O'Branski, Erin E ^a   Ware, Russell E ^a  

^a DUKE UNIVERSITY MEDICAL CENTER   (United States)

Author keywords

Children; Erythorcytes; F cells; Fetal hemoglobin; Sickle cell disease

Indexed keywords

HEMOGLOBIN F;

ADOLESCENT; ARTICLE; CHILD; CONTROLLED STUDY; ERYTHROCYTE; FEMALE; HUMAN; MAJOR CLINICAL STUDY; MALE; PRIORITY JOURNAL; SICKLE CELL ANEMIA;

ADOLESCENT; ADULT; ANEMIA, SICKLE CELL; CHILD; CHILD, PRESCHOOL; FEMALE; FETAL HEMOGLOBIN; GENOTYPE; HEMOGLOBIN C; HEMOGLOBIN, SICKLE; HUMANS; MALE;

EID: 0031012328     PISSN: 03618609     EISSN: None     Source Type: Journal    
DOI: 10.1002/(SICI)1096-8652(199701)54:1<40::AID-AJH6>3.0.CO;2-4     Document Type: Article

References (40)

1. Platt OS, Dover GJ: Sickle cell disease. In Nathan DG, Oski FA (eds): "Hematology of Infancy and Childhood." Philadelphia: W.B. Saunders, 1993, pp 732-782.
2. Charache S, Lubin B, Reid CD (eds): "Management and Therapy of Sickle Cell Disease." 1991.
3. Platt OS, Thorington BD, Brambilla DJ, Milner PF, Rosse WF, Vichinsky E, Kinney TR: Pain in sickle cell disease. N Engl J Med 325:11, 1991.
4. Powars DR: β8-gene-cluster haplotypes in sickle cell anemia. Hematol Oncol Clin North Am 5:475, 1991.
5. Steinberg MH, Hsu H, Nagel RL, Milner PF, Adams JG, Benjamin L, Kryd S, Gillette P, Gilman J, Josifovska O, Hellman-Erlingsson S, Safaya S, Huey L, Rieder RF: Gender and haplotype effects upon hematological manifestations of adult sickle cell anemia. Am J Hematol 48:175, 1995.
6. Steinberg MH, Rosenstock W, Coleman MB, Adams JG, Platica O, Cedenp M, Rieder RF, Wilson JT, Milner P, West S: The effects of alpha thalassemia and microcytosis on the hematologic and vaso-occlusive severity of sickle cell anemia. Blood 63:1353, 1984.
7. Baum KF, Dunn DT, Maude GH, Serjeant G: The painful crisis of homozygous sickle cell disease: A study of risk factors. Arch Intern Med 147:1231, 1987.
8. s-gene-cluster haplotypes as prognostic indicators of vital organ failure. Semin Hematol 28:202, 1991.
9. Phillips G, Coffey B, Tran-son-tay R, Kinney TR, Orringer EP, Hochmuth RM: Relationship of clinical severity to packed cell rheology in sickle cell anemia. Blood 78:2735, 1991.
10. Platt OS, Brambilla DJ, Rosse WF, Milner PF, Castro O, Steinberg MH, Klug PP: Mortality in sickle cell disease. N Engl J Med 330:1939, 1994.
11. Charache S: Fetal hemoglobin, sickling, and sickle cell disease. Adv Pediatr 37:1, 1990.
12. Wood WG: Increased HbF in adult life. Baillieres Clin Haematol 6:177, 1993.
13. Serjeant GR: Foetal haemoglobin in homozygous sickle cell disease. Clin Haematol 4:109, 1975.
14. Wood WG, Stamatoyannopoulos G, Lim G, Nute PE: F-cells in the adult: Normal values and levels in individuals with hereditary and acquired elevations of HbF. Blood 46:671, 1975.
15. Rucknagel DL, Sarniak SA, Whitten CF, Odenheimer DA: Fetal hemoglobin concentration predicts disease severity in children with sickle cell anemia. In Stamatoyannopoulos G, Nienhuis AW (eds): "Progress in Clinical and Biological Research, 251: Developmental Control of Globin Gene Expression." New York: Alan R. Liss, 1987, p 487.
16. Odenheimer DJ, Sarnaik SA, Whitten CF, Rucknagel DL, Sing CF: The relationship between fetal hemoglobin and disease severity in children with sickle cell anemia. Am J Med Genet 27:525, 1987.
17. 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.
18. Powars DR, Weiss JN, Chan LS, Schroeder WA: Is there a threshold of fetal hemoglobin that ameliorates morbidity in sickle cell anemia? Blood 63:921, 1984.
19. Leikin SL, Gallagher D, Kinney TR, Sloane D, Klug P, Rida W: Mortality in children and adolescents with sickle cell disease. Pediatrics 84:500, 1989.
20. Miyoshi F, Kaneto Y, Kawai H, Ohchi H, Niki S, Hasegawa K, Shirakami A, Yamano T. X-linked dominant control of F-cells in normal adult life: Characterization of the Swiss type as hereditary persistence of fetal hemoglobin regulated dominantly by gene(s) on X chromosome. Blood 72:1854, 1988.
21. s haplotypes in homozygous sickle cell disease. Eur J Haematol 45:1, 1990.
22. Chang YC, Smith KD, Moore RD, Serjeant G, Dover GJ: An analysis of fetal hemoglobin variation in sickle cell disease: The relative contributions of the X-linked factor, β-globin haplotypes, α-globin gene number, gender, and age. Blood 85:1111, 1995.
23. Dover GJ, Boyer SH, Charache S, Heintzelman K: Individual variation in the production and survival of F cells in sickle-cell disease. N Engl J Med 299:1428, 1978.
24. Boyer SH, Belding TK, Margolet L, Noyes AN: Fetul hemoglobin restriction to a few erythrocytes (F cells) in normal human adults. Science 188:361, 1975.
25. Kinney TR, Sawtschenko M, Whorton M, Shearin J, Stine C, Hofman L, Safko R, Vitaglione T, Kaufman RE: Techniques' comparison and report of the North Carolina experience. Pediatrics 83(suppl):843, 1989.
26. Dover GJ, Boyer SH: Fetal hemoglobin-containing cells have the same mean corpuscular hemoglobin as cells without fetal hemoglobin: A reciprocal relationship between gamma- and beta-globin gene expression in normal subjects and in those with high fetal hemoglobin production. Blood 69:1109, 1987.
27. Brittenham GM, Schechter AN, Noguichi CT: Hemoglobin S polymerization: Primary determinant of the hemolytic and clinical severity of the sickling syndromes. Blood 65:183, 1985.
28. Noguchi CT, Rodgers GP, Serjeant G, Schechter AN: Levels of fetal hemoglobin necessary for treatment of sickle cell disease. N Engl J Med 318:96, 1988.
29. Bookchin RM, Nagel RL, Balazs T: Role of hybrid tetramer formation in gelation of haemoglobin S. Nature 256:667, 1975.
30. Singer K, Singer L: The gelling phenomenon of sickle cell hemoglobin: Its biologic and diagnostic significance. Blood 8:1008, 1953.
31. Bertles JF, Rabinowtiz R, Dobler J: Hemoglobin interaction: Modification of solid phase composition in the sickling phenomenon. Science 169:375, 1970.
32. 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.
33. 2 on the polymerization of hemoglobin S at physiologic ligand saturations. Proc Natl Acad Sci USA 90:5039, 1993.
34. Benesch RE, Edalji R, Benesch R, Kwong S: Solubilization of hemoglobin S by other hemoglobins. Proc Natl Acad Sci USA 77:5130, 1988.
35. Shepard MK, Weatherall DJ, Conley CL: Semi-quantitative estimation of the distribution of fetal hemoglobin in red cell populations. Bull J Hopkins Hosp 110:293, 1962.
36. Papayannopoulou T, Vichinsky E, Stamatoyannopoulos G: Fetal Hb production during acute erythroid expansion. Observations in patients with transient erythroblastopenia and post-phlebotomy. Br J Haematol 44:535, 1980.
37. Schiliro G, Russo A, Romeo MA, Testa R, Musumeci S, Mancuso GR, Russo G: Increase of F cells during acute hemolysis in glucose-6-phosphate dehydrogenase-deficient males. Pediatr Res 19:397, 1985.
38. Goldberg MA, Brugnara C, Dover GJ, Schapira L, Charache S, Bunn HF: Treatment of sickle cell anemia with hydroxyurea and erythropoietin. N Engl J Med 323:366, 1990.
39. Charache S, Dover GJ, Moore RD, Eckert S, Ballas SK, Koshy M, Milner PFA, Orringer EP, Phillips G, Platt OS, Thomas GH: Hydroxyurea: Effects on hemoglobin F production in patients with sickle cell anemia. Blood 79:2555, 1992.
40. Charache S, Terrin ML, Moore RD, Dover GJ, Barton RB, Eckert SV, McMahon RP, Bonds DR: Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 332:1317, 1995.