degree of morbidity, moderate to severe hemolytic anemia among vulnerable segments of the society like infants and children, adolescent girls,

pregnant women, etc. and several deaths in India.The cumulative gene frequency of the three most predominant abnormal hemoglobins, i.e. sickle

hemoglobin, hemoglobin D and hemoglobin E has been found to be 5.35% in India. Prevalence of sickle gene is found to be 0–18% in North eastern

India, 0–33.5% in Western India, 22.5–44.4% in Central India and 1–40% in Southern India. Average prevalence of sickle cell carrier among the

tribal population was 10%. The sickling disorders– HbSS, HbSD, HbSE, HbS/ β– thalassemia and other compound heterozygous

hemoglobinopathies are all clinically significant, as these combinations present with different manifestations and degrees of severity, making

precise identification important. Automated cation–exchange High Performance Liquid Chromatography (HPLC) has emerged as an excellent

screening tool for diagnosing these abnormal hemoglobins/ thalassemic states.

Aims and Objective: 1) To study clinical features, hematological features and HPLC findings in patients of compound heterozygous hemoglobin

disorders. 2) To generate data regarding ethnicity and consanguinity in cases of compound heterozygous hemoglobin disorders.3) To evaluate the

role of family study in cases of compound heterozygous hemoglobin disorders.

Material and Methods: This is a study of double heterozygosity by High Performance Liquid Chromatography (HPLC). The study was carried

out in Department of Pathology, RIMS, Ranchi, after the approval of the college ethical committee. Study design: The present study is a Cross

sectional descriptive study. Study period: Conducted from July 2013 to August 2014.

Observation and result: In the present study, total 70 cases of compound/ double heterozygous state were studied in the Department of Pathology,

Rajendra Institute of Medical Sciences, Ranchi from July 2013 to August 2014. The compound heterozygous states are grouped as Group A– Sβ

thalassemia – 22+38=60 cases (85.71%), Group B– Eβ thalassemia – 6+4=10 cases (14.28%). 54.28% cases showed consanguinity. This highlights

the role of premarital counseling. Scheduled Tribe (ST) (37.14%) and Muslim (27.14%) were the most commonly affected ethnic groups, this

emphasizes on the need to formulate community based studies and implement on the strategies accordingly. Pallor was the most common feature

among all the groups followed by jaundice and splenomegaly. Anemia, jaundice and splenomegaly should be investigated in detail for the

possibility of Hb disorder.

Conclusion: The definite identification of disorders of hemoglobin synthesis can be achieved only by DNA analysis, but, in the Indian scenario,

family studies on HPLC might be useful as there is paucity of funds, and facilities for DNA analysis are not readily available. Family study is an

equally efficacious and cost effective tool. This study is carried out to correctly diagnose the compound heterozygous states by performing HPLC

and family studies if possible, and to generate data so that health care resources can be successfully planed and targeted at them.