Soluble transferrin receptor in sickle cell diseases

correlation with spleen function

Authors

  • Helena Zerlotti Wolf Grotto Universidade Estadual de Campinas
  • Elza Miyuki Kimura Universidade Estadual de Campinas
  • Márcia Victor Carneiro Universidade Estadual de Campinas

Keywords:

Hemoglobinopathies, Pitted erythrocytes, Red cell ferritin, Soluble transferrin receptor, Sickle cell disease

Abstract

OBJECTIVE: To correlate spleen function with soluble transferrin receptor (sTfR) levels and red cell ferritin (RCF) values in patients with sickle cell diseases. DESIGN: Prospective study. LOCATION: University Hospital, School of Medical Sciences, State University of Campinas; a tertiary hospital. PARTICIPANTS: 60 patients with sickle cell diseases, in a steady state, who had not received blood transfusions for 3 months; 28 normal individuals with no clinical or laboratory signs of anemia. MEASUREMENTS: Determination of serum iron, transferrin iron-binding capacity, serum ferritin, RCF and sTfR. Evaluation of spleen function: erythrocytes with pits were quantified. RESULTS: Patients with sickle cell anemia had sTfR levels significantly higher than in normal individuals or those with HbSC (p=0.0001) and there was an inverse correlation between sTfR and fetal Hb (p=0.0016). RCF values were significantly higher in sickle cell anemia patients than in normal individuals or those with HbSC (p=0.0001), and there was a correlation between RCF and pitted erythrocytes (p=0.0512). CONCLUSION: The association between sTfR and fetal Hb confirms the contribution of fetal Hb to improving the hemolytic state by minimizing the consequent reactive erythrocyte expansion. High sTfR levels are not related to the degree of spleen function deficiency seen in sickle cell disease patients. The deficiency in the exocytosis process of the spleen occurring in sickle cell anemia patients may contribute to their accumulation of RCF.

Downloads

Download data is not yet available.

Author Biographies

Helena Zerlotti Wolf Grotto, Universidade Estadual de Campinas

MD, PhD. Department of Clinical Pathology, School of Medical Sciences, State University of Campinas (UNICAMP), Campinas, SP, Brazil.

Elza Miyuki Kimura, Universidade Estadual de Campinas

Biologist, Department of Clinical Pathology, School of Medical Sciences, State University of Campinas (UNICAMP), Campinas, SP, Brazil.

Márcia Victor Carneiro, Universidade Estadual de Campinas

Biologist, Department of Clinical Pathology, School of Medical Sciences, State University of Campinas (UNICAMP), Campinas, SP, Brazil.

References

Kohgo Y, Nishisato T, Kondo H, Tsushima N, Niitsu Y. Circulating transferrin receptor in human serum. Br J Haematol 1986;64:277-81.

Skikne BS, Flowers CH, Cook JD. Serum transferrin receptor: a quantitative measure of tissue iron deficiency. Blood 1990;75:1870-6.

Baynes RD, Cook JD, Bothwell TH, Friedman BM, Meyer TE. Serum transferrin receptor in hereditary hemochromatosis and African siderosis. Am J Hematol 1994;45:288-92.

Huebers HA, Beguin Y, Pootrakul P, Einspahr D, Finch CA. Intact transferrin receptors in human plasma and their relation to erythropoiesis. Blood 1990;75:102-7.

Singhal A, Cook JD, Skikne BS, Thomas P, Serjeant B, Serjeant G. The clinical significance of serum transferrin receptor levels in sickle cell disease. Br J Haematol 1993;84:301-4.

Galanello R, Barella S, Turco MA, et al. Serum erythropoietin and erythropoiesis in high- and low-fetal hemoglobin ß-thalassemia intermedia patients. Blood 1994;83:561-5.

Pearson HA, Gallagher D, Chilcote R, et al. Developmental pattern of splenic dysfunction in sickle cell disorders. Pediatrics 1985;76:392-7.

Jacobs A, Peters SW, Bauminger ER, Eikelboom J, Ofer S, Rachmilewitz EA. Ferritin concentration in normal and abnormal erythrocytes measured by immunoradiometric assay with antibodies to heart and spleen ferritin and Mössbauer spectroscopy. Br J Haematol 1981;49:201-7.

Rogers DW, Serjeant BE, Serjeant GR. Early rise in "pitted" red cell count as a guide to susceptibility to infection in childhood sickle cell anaemia. Arch Dis Child 1982;57:338-42.

Pembrey ME, MacWade P, Weatherall DJ. Reliable routine estimation of small amounts of foetal haemoglobin by alkali denaturation. J Clin Pathol 1972;25:738-40.

Peters SW, Jacobs A, Fitzsimons E. Erythrocyte ferritin in normal subjects and patients with abnormal iron metabolism. Br J Haematol 1983;53:211-6.

Grotto HZW, Costa FF. Pattern of splenic phagocytic function in Brazilian patients with sickle cell disease. Rev Paul Med 1992;110:262-6.

Bauminger ER, Cohen SG, Ofer S, Rach-Milevitz EA. Quantitative studies of ferritin-like iron in erythrocytes of thalassemia, sickle cell anemia and Hb Hammersmith with Mössbauer spectroscopy. Proc Natl Acad Sci USA 1979;76:939-43.

Musto P, Lombardi G, Centra M, Modoni S, Carotenuto M, DiGiorgio G. Soluble transferrin receptor in beta-thalassaemia. Lancet 1993;342:1058.

Cook JD, Skikne BS, Baynes RD. Serum transferrin receptor. Annu Rev Med 1993;44:63-74.

Serjeant G, Serjeant B, Stephens A, Roper D, Higgs D, Beckford M, Cook J, Thomas P. Determinants of haemoglobin level in steady-state homozygous sickle cell disease. Br J Haematol 1996;92:143-9.

Beguin Y. The soluble transferrin receptor: biological aspects and clinical usefulness as quantitative measure of erythropoiesis. Haematologica 1992;77:1-10.

Cazzola M, Beguin Y. New tools for clinical evaluation of erythron function in man. Br J Haematol 1992;80:278-84.

Ferguson BJ, Skikne BS, Simpson KM, Baynes RD, Cook JD. Serum transferrin receptor distinguishes the anemia of chronic disease from iron deficiency anemia. J Lab Clin Med 1992;19:385-90.

Scherezenmeier H, Noé G, Raghavachar A, Rich IN, Heimple H, Kubanek B. Serum erythropoietin and serum transferrin receptor levels in aplastic anaemia. Br J Haematol 1994;88:286-94.

Casper JT, Koethe S, Rodey GE, Thatcher G. A new method for studying splenic reticuloendothelial dysfunction in sickle cell disease patients and its clinical application: a brief report. Blood 1976;47:183-8.

Sills RH. Splenic function in children with hemoglobin SC and sickle ß thalassemia. J Natl Med Assoc 1983;75:991-4.

Crosby WH. Hematopoiesis in the human spleen. Arch Intern Med 1983;143:1321-2.

Downloads

Published

1999-07-07

How to Cite

1.
Grotto HZW, Kimura EM, Carneiro MV. Soluble transferrin receptor in sickle cell diseases: correlation with spleen function. Sao Paulo Med J [Internet]. 1999 Jul. 7 [cited 2025 Mar. 15];117(4):145-50. Available from: https://periodicosapm.emnuvens.com.br/spmj/article/view/2542

Issue

Section

Original Article