The Effect of Blood Transfusion on Growth of Patients with Hb E/β-Thalassemia

Abstract

A retrospective evaluation of growth in 112 patients (68 males, 44 females) with Hb E (HBB: c.79G>A)/β-thalassemia (β-thal), classified as 88 transfusion-dependent thalassemia (TDT) and 24 non transfusion-dependent thalassemia (NTDT), is reported. Patients with TDT have received regular transfusions of red blood cells (RBCs) 15 mL/kg every 4 weeks to maintain pre transfusion hemoglobin (Hb) levels of at least 9.0 g/dL and were categorized according to age at initiation of regular RBC transfusion as subgroup 1, <4 years; subgroup 2, 4–10 years, and subgroup 3, >10 years. Iron chelation was initiated at the mean age of 7 years. The results revealed that patients in subgroups 1 and 2, receiving RBC transfusions at a young age (2.9 and 6.9 years, respectively), had normal prepubertal growth at enrollment and last follow-up. Patients in subgroup 3, with the lowest initial height Z-score of −2.10, were able to achieve comparable final adult height as those in subgroups 1 and 2. The mean final height of 21 males and 13 females with TDT at the ages of 18.9 and 18.7 years was 168.1 and 157.7 cm, respectively, which did not significantly differ from their midparental height and those with NTDT. Early initiation of optimal transfusion and iron chelation promoted normal prepubertal growth. However, delayed initiation of transfusion at age 12 years impaired prepubertal growth but they could achieve normal final adult height.

Keywords:

• Final height
• growth
• Hb E/β-thalassemia (Hb E/β-thal)

Acknowledgments

The authors acknowledge all children, adolescents, and caregivers who participated in the study and Professor Pat Mahachoklertwattana (Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand) for the helpful endocrinological interpretation.

Author contributions

A. Chuansumrit, N. Sirachainan, P. Kitpoka and D. Songdej performed analysis and interpretation; A. Chuansumrit, N. Sirachainan and P. Wongwerawattanakoon, were involved in blood collection; W. Sasanakul and P. Kadegasem analyzed laboratory results. All authors gave final approval of the version to be published.

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.