Publication: A prospective analysis for prevalence of complications in Thai nontransfusion-dependent Hb E/β-thalassemia and α-thalassemia (Hb H disease)
Issued Date
2018-05-01
Resource Type
ISSN
10968652
03618609
03618609
Other identifier(s)
2-s2.0-85042045296
Rights
Mahidol University
Rights Holder(s)
SCOPUS
Bibliographic Citation
American Journal of Hematology. Vol.93, No.5 (2018), 623-629
Suggested Citation
Supachai Ekwattanakit, Noppadol Siritanaratkul, Vip Viprakasit A prospective analysis for prevalence of complications in Thai nontransfusion-dependent Hb E/β-thalassemia and α-thalassemia (Hb H disease). American Journal of Hematology. Vol.93, No.5 (2018), 623-629. doi:10.1002/ajh.25046 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/46701
Research Projects
Organizational Units
Authors
Journal Issue
Thesis
Title
A prospective analysis for prevalence of complications in Thai nontransfusion-dependent Hb E/β-thalassemia and α-thalassemia (Hb H disease)
Other Contributor(s)
Abstract
© 2018 Wiley Periodicals, Inc. Recently, complications in patients with nontransfusion-dependent thalassemia (NTDT), in particular those with β-thalassemia intermedia (β-TI), were found to be significantly different from those in patients with transfusion dependent thalassemia (TDT), mainly β-thalassemia major (β–TM). However, this information is rather limited in other forms of NTDT. In this prospective study, adult Thai NTDT patients were interviewed and clinically evaluated for thalassemia related complications. Fifty-seven NTDT patients (age 18-74 years), 59.6% Hb E/β-thalassemia and 40.4% Hb H disease, were recruited; 26.4% were splenectomized. The most common complications were gallstones (68.4%), osteoporosis (26.3%), and pulmonary hypertension (15.8%). Splenectomy was associated with higher rate of gallstones and serious infection (P =.001 and.052, respectively), consistent with a multivariate analysis (RR = 9.5, P =.044, and RR = 15.1, P =.043, respectively). In addition, a higher hemoglobin level was inversely associated with gallstones in both univariate and multivariate analyses (P =.01 and.022, respectively). Serum ferritin was associated with abnormal liver function (P =.002). In contrast to the previous study, the prevalence of thrombosis was less common in our population (1.7%), probably due to differences in transfusion therapy, ethnicity, and underlying genotypes. For the first time, this prospective study provided the current prevalence of NTDT related complications in a Southeast Asian population with a different underlying genetic basis compared with previous studies. Although individual prevalence of each complication might differ from other studies, several important clinical factors such as splenectomy, degree of anemia, and iron overload seem to be determining risks of developing these complications consistently across different ethnicities.