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Erythropoietin activity in the serum of beta thalassemic patients
Author(s) -
Manor Daphna,
Fibach Eitan,
Goldfarb Ada,
Rachmilewitz Eliezer A.
Publication year - 1986
Publication title -
scandinavian journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0036-553X
DOI - 10.1111/j.1600-0609.1986.tb02301.x
Subject(s) - ineffective erythropoiesis , thalassemia , beta thalassemia , medicine , chelation therapy , erythropoietin , anemia , erythropoiesis , endocrinology , splenectomy , hemolytic anemia , hemoglobinopathy , beta (programming language) , transferrin , immunology , spleen , computer science , programming language
Serum erythropoietic activity was determined in 32 patients with beta thalassemia major and intermedia. Quantitation was performed by an in vitro bioassay using rabbit erythroid precursor cells (CFU‐E) either by colony assay or by 3 H‐thymidine uptake. 20 polytransfused beta‐thalassemic major patients had erythropoietic activity (mean 89.3 ± 36 milliunits/ml) which was not significantly different (p > 0.2) from normal individuals (51.3 ± 32 milliunits/ml). 12 untransfused patients with beta thalassemia intermedia were found to have comparable serum erythropoietic activity (p > 0.1). These levels were much lower than those found in patients with aplastic anemia who had a comparable degree of anemia. We have shown that the low EPO activity in thalassemic patients was not due to experimental conditions (excess of ferritin, low transferrin) nor to specific inhibitors appearing in this disease. No correlation was found between the erythropoietic activity and sex or other clinical parameters of the patients such as severity of the anemia, splenectomy, iron chelation or transfusion therapy. 4 young thalassemic children (1–2 yr of age) studied had high erythropoietic activity ranging from 661 to 5793 milliunits/ml ‐ significantly different from normal children of the same age. It is suggested, therefore, that a decrease in serum erythropoietin levels develops during the course of the disease.