Ineffective erythropoiesis plays a well established role in the pathophysiology of disease expression in β-thalassemia major and intermedia. CD177 expression was investigated in different clinical conditions. The study aimed to analyze neutrophil expression of CD177 in β-thalassemia patients and its correlation with serum soluble transferrin receptor (s-TfR) concentration as a marker for the extent of erythropoiesis and hence disease severity in these patients. Flow cytometric analysis of neutrophil CD177 expression and enzyme immunoassay measurement of serum s-TfR concentration were assessed in 45 β-thalassemia patients of whom 36 had β-thalassemia major and nine had β-thalassemia intermedia. They were also assessed in 21 age- and gender-matched control children. Neutrophil mean fluorescence intensity ratio (MFIR) of CD177 expression was significantly higher in patients than in controls (p < 0.001). There was highly significant increase in serum s-TfR concentration in β-thalassemia patients than in controls (p < 0.001). There was a highly significant positive correlation between MFIR of CD177 expression and serum s-TfR concentration in β-thalassemia patients (r = 0.59, p < 0.001). Elevated CD177 expression is not only a specific feature of polycythemia rubra vera (PV) but may be also an indicator of increased erythropoietic activity in thalassemia syndromes.
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Montaser et al. (2011) studied this question.
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