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Erythroid marrow activity and hemoglobin H levels in hemoglobin H disease.
J Pediatr Hematol Oncol. 1998 Nov-Dec; 20(6):539-44.JP

Abstract

PURPOSE

To determine serum immunoreactive erythropoietin (Epo) and soluble transferrin receptors (sTfR) levels in patients with hemoglobin H (HbH) disease and the correlation with HbH levels and alpha-globin genotype.

PATIENTS AND METHODS

Twenty patients with HbH disease were studied. Methods applied included cation-exchange high pressure liquid chromatography for HbH levels, chemoluminescence for Epo concentration, immunoassay for sTfR concentration, and DNA analysis for alpha-globin genotype characterization.

RESULTS

Serum Epo and sTfR levels were significantly elevated (46.6+/-26.8 IU/l and 5.6+/-1.8 mg/l, respectively) in patients with HbH disease compared to controls (9.2+/-3.3 IU/l and 1.8+/-0.7 mg/l, respectively). Epo and sTfR levels correlated positively with HbH concentration (r = 0.93 and 0.80, respectively). The highest Epo and sTfR values were observed in three patients with the highest HbH levels who all had nondeletion alpha-thalassemia mutations.

CONCLUSION

Epo and sTfR levels are increased in patients with HbH disease; this increase is directly related to the HbH concentration that usually reflects the degree of globin polypeptide imbalance. The correlation of Epo, sTfR, and reticulocyte production index in these patients indicates that anemia in HbH disease mainly is caused by ineffective erythropoiesis and a mild degree of peripheral hemolysis.

Authors+Show Affiliations

Hematology Laboratory, Aghia Sophia Children's Hospital, Athens, Greece.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

9856674

Citation

Papassotiriou, I, et al. "Erythroid Marrow Activity and Hemoglobin H Levels in Hemoglobin H Disease." Journal of Pediatric Hematology/oncology, vol. 20, no. 6, 1998, pp. 539-44.
Papassotiriou I, Traeger-Synodinos J, Kanavakis E, et al. Erythroid marrow activity and hemoglobin H levels in hemoglobin H disease. J Pediatr Hematol Oncol. 1998;20(6):539-44.
Papassotiriou, I., Traeger-Synodinos, J., Kanavakis, E., Karagiorga, M., Stamoulakatou, A., & Kattamis, C. (1998). Erythroid marrow activity and hemoglobin H levels in hemoglobin H disease. Journal of Pediatric Hematology/oncology, 20(6), 539-44.
Papassotiriou I, et al. Erythroid Marrow Activity and Hemoglobin H Levels in Hemoglobin H Disease. J Pediatr Hematol Oncol. 1998 Nov-Dec;20(6):539-44. PubMed PMID: 9856674.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Erythroid marrow activity and hemoglobin H levels in hemoglobin H disease. AU - Papassotiriou,I, AU - Traeger-Synodinos,J, AU - Kanavakis,E, AU - Karagiorga,M, AU - Stamoulakatou,A, AU - Kattamis,C, PY - 1998/12/18/pubmed PY - 1998/12/18/medline PY - 1998/12/18/entrez SP - 539 EP - 44 JF - Journal of pediatric hematology/oncology JO - J Pediatr Hematol Oncol VL - 20 IS - 6 N2 - PURPOSE: To determine serum immunoreactive erythropoietin (Epo) and soluble transferrin receptors (sTfR) levels in patients with hemoglobin H (HbH) disease and the correlation with HbH levels and alpha-globin genotype. PATIENTS AND METHODS: Twenty patients with HbH disease were studied. Methods applied included cation-exchange high pressure liquid chromatography for HbH levels, chemoluminescence for Epo concentration, immunoassay for sTfR concentration, and DNA analysis for alpha-globin genotype characterization. RESULTS: Serum Epo and sTfR levels were significantly elevated (46.6+/-26.8 IU/l and 5.6+/-1.8 mg/l, respectively) in patients with HbH disease compared to controls (9.2+/-3.3 IU/l and 1.8+/-0.7 mg/l, respectively). Epo and sTfR levels correlated positively with HbH concentration (r = 0.93 and 0.80, respectively). The highest Epo and sTfR values were observed in three patients with the highest HbH levels who all had nondeletion alpha-thalassemia mutations. CONCLUSION: Epo and sTfR levels are increased in patients with HbH disease; this increase is directly related to the HbH concentration that usually reflects the degree of globin polypeptide imbalance. The correlation of Epo, sTfR, and reticulocyte production index in these patients indicates that anemia in HbH disease mainly is caused by ineffective erythropoiesis and a mild degree of peripheral hemolysis. SN - 1077-4114 UR - https://www.unboundmedicine.com/medline/citation/9856674/Erythroid_marrow_activity_and_hemoglobin_H_levels_in_hemoglobin_H_disease_ L2 - https://Insights.ovid.com/pubmed?pmid=9856674 DB - PRIME DP - Unbound Medicine ER -