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Background Thalassemia and hemoglobinopathies are the
most common inherited disorders in many areas of the world,
including South East Asia. The siblings of thalassemia major is a
group of high risk to carry the gene of thalassemia. Determining
the carrier is useful for early treatment planning and prevention
to the next child.
Objective To determine carrier status among siblings of
thalassemia patients using a capillary electrophoresis system.
Methods A cross-sectional study on the siblings of thalassemia
major patients was performed from January 2011 to February
2012 at Dr. Moewardi Hospital. Complete blood counts were
performed in the siblings. Subjects with mean corpuscular volume
(MCV) <80 fl and mean corpuscular hemoglobin (MCH) <27
pg were subjected to analize hemoglobin fraction by capillary
Results Of the 26 subjects, there were 12 males and 14 females.
The mean age was 9.38 (SD 6.8) years (range 1 to 29 years). From
the siblings, 10 were identified as normal, 5 were identified as ß
thalassemia carriers and 5 were hemoglobin E (HbE) carriers. Six
siblings were diagnosed with ß thalassemia/ HbE.
Conclusion There are high occurrence of the two common types
of thalassemia carriers (ß and HbE) in our small group of subjects
who had a family history of thalassemia. Most of the siblings
of thalassemia had low MCV and MCH.
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2. Fucharoen G, Fucharoen S, Sanchaisuriya K, Sae-Ung N, Suyasunanond A, Sriwilai P, et al. Frequency distribution and haplotypic heterogeneity of ßE-globin gene among eight minority groups of Northeast Thailand. Hum Hered. 2002;53:18-22.
3. Lookopoulos D, Kollia P. Worldwide distribution of a thalassaemia: In: Steinberg MH, Forget B, Higgs DR, Nagel RL. Disorder of haemoglobin, genetic, pathophysiology, and clinical management. Cambridge: Cambridge University Press; 2001. p. 861-77.
4. Wahidiyat I. Thalassemia dan permasalahannya di Indonesia. Sari Pediatri. 2003;5:2-3.
5. Beyan C, Kaptan K, Irfan A. Predictive value of discrimination indices in differential diagnosis of iron deficiency anemia and beta-thalassemia trait. Eur J Haematol. 2007;78:524-6.
6. Ahmed MG, Fahim F. Red blood cell indices in thalassemias. Haematology Update. 2011.
7. Fucharoen G, Sanchaisuriya K, Sae-ung N, Dangwibul S, Fucharoen S. A simplified screening strategy for thalassaemia and haemoglobin E in rural communities in south-east Asia. Bull World Health Organ. 2004;82:364-72.
8. Cotton F, Lin C, Fontaine B, Gulbis B, Janssens J. Evaluation of a capillary electrophoresis method for routine determination of hemoglobins A2 and F. Clin Chem. 1999;45:237-43.
9. Cao A, Galanello R, Rosatelli MC. Prenatal diagnosis and screening of the haemoglobinopathies. Baillieres Clin Haematol. 1998;11:215-38.
10. Hussain Z, Malik N, Chughtai AS. Diagnostic significance of red cell indices in beta thalassaemia trait. Biomedica. 2005;21:129-31.
11. Goldbloom RB. Screening for hemoglobinopathies in Canada. In: The Canadian Task Force on the periodic health hxamination. The Canadian guide to clinical preventive health care. Ottawa: Public Health Agency of Canada; 1994. p. 206-18
12. Louhabi A, Philippe M, Lali S. Evaluation of a new Sebia kit for analysis of hemoglobin fractions and variants on the Capillarys system. Clin Chem Lab Med. 2006;44:340-5.
13. Sangkitporn S, Sangkitporn SK, Tanjatham S, Suwannakan B, Rithapirom S, et al. Multicenter validation of fully automated capillary electrophoresis method for diagnosis of thalassemias and hemoglobinopathies in Thailand. Southeast Asian J Trop Med Public Health. 2011;42:1224-32.
14. Angastiniotis M, Modell B, Englezos P, Boulyjenkov V. Prevention and control of haemoglobinopathies. Bull World Health Organ. 1995;73:375-86.