Abstract:
:To evaluate the association between fetal hemoglobin (HbF) levels and morbidity in β-thalassemia intermedia (TI), we analyzed data from 63 untransfused patients who had also never received HbF induction therapy. Patient records were reviewed for any history of 10 predefined morbidities. Laboratory measurements for markers of ineffective erythropoiesis were also obtained. The mean age of patients was 32.1 years, 47.6% were males, and the median HbF level was 37.2%. HbF levels correlated positively with total hemoglobin, yet negatively with growth differentiation factor-15 and non-transferrin-bound iron levels. Median HbF levels were significantly lower in patients with the majority of evaluated morbidities than in those without. There was a strong negative adjusted linear correlation between the HbF level and the total number of morbidities (R(2) = 0.825, P < .001). The HbF threshold of 63.7% had 95.5% sensitivity and 100% specificity for ensuring absence of morbidity. There exists a strong association between HbF levels and morbidity in the subset of untransfused patients with TI.
journal_name
Bloodjournal_title
Bloodauthors
Musallam KM,Sankaran VG,Cappellini MD,Duca L,Nathan DG,Taher ATdoi
10.1182/blood-2011-09-382408subject
Has Abstractpub_date
2012-01-12 00:00:00pages
364-7issue
2eissn
0006-4971issn
1528-0020pii
blood-2011-09-382408journal_volume
119pub_type
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