Abstract
BackgroundHemoglobin S and E are commonly occurring hemoglobin variants among distinctly separate tribal populations of Central and Northeast India, respectively. Combined heterozygosity for hemoglobin S and E or hemoglobin SE disease is a benign clinical condition with rare incidence. Reports of approximately 46 hemoglobin SE cases are available worldwide. We conducted a screening program to study the prevalence of hemoglobin variants among the tribal population working in the tea estates of Northeast India. A total of 551 subjects were screened, and complete blood count was performed. Based on their hematological profiles, hemoglobin typing was done for 218 subjects.Case presentationWe describe a case of an adolescent male of Munda tribe diagnosed as double heterozygous for hemoglobin S and E. On screening of the nuclear family of the subject, the mother was found to have hemoglobin E disease and father as hemoglobin S trait. Both siblings of the subject were diagnosed as hemoglobin E trait.ConclusionThis is the first case of compound heterozygous for hemoglobin S and E to be reported from the tea tribes of Assam, India.
Highlights
Hemoglobin S and E are commonly occurring hemoglobin variants among distinctly separate tribal populations of Central and Northeast India, respectively
This is the first case of compound heterozygous for hemoglobin S and E to be reported from the tea tribes of Assam, India
Such double heterozygous condition was reported from a 15-year-old Teli male (Other Backward Caste) from Central India who complained of frequent upper respiratory tract infections, weakness, and fatigue [11]
Summary
Hemoglobinopathies are monogenic disorders characterized by abnormal hemoglobin structure [1]. A large workforce is employed in the tea estates of Assam, which constitutively forms the tea-tribe community These laborers were brought to Assam by British rulers from the present states of Chhattisgarh, West Bengal, Odisha, and Jharkhand during the last part of the nineteenth century. We conducted a study to investigate the prevalence of sickle cell disorder among the tea estate laborers of Udalguri District of Assam, India. The case subject showed low Hb (7.8 g/dl), MCHC (29.3%), and RBC count (2.34 million/mm3) Hb: hemoglobin; RBC: red blood cell; MCV: mean corpuscular volume; MCH: mean corpuscular hemoglobin; MCHC: mean corpuscular hemoglobin concentration; RDW: red cell distribution width
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