Hemoglobin profiles of siblings of thalassemia patients

Abstract

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
electrophoresis.
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.