title: Hb SE disease: a clinico-hematological profile.
abstract: Hb S and Hb E are globally common hemoglobinopathies. However, Hb SE double heterozygous state is uncommon, with only 25 cases reported so far in literature. We present two more cases. One presented with gallstones, and the other was asymptomatic. This type of disorder was previously described as a relatively asymptomatic condition compared to HbSS. A review of the 25 reported cases in literature shows that 40.7% (11/27) of these cases are symptomatic. Gender, hematological parameters and levels of Hb S, E or F do not predict clinical severity.
authors.affiliation: Department of Hematology, All-India Institute of Medical Sciences, New Delhi, India.
authors.forename: Pravas
authors.initials: P
authors.lastname: Mishra
authors.forename: H P
authors.initials: HP
authors.lastname: Pati
authors.forename: Tathagat
authors.initials: T
authors.lastname: Chatterjee
authors.forename: Ashish
authors.initials: A
authors.lastname: Dixit
authors.forename: Dharma R
authors.initials: DR
authors.lastname: Choudhary
authors.forename: M Upendra
authors.initials: MU
authors.lastname: Srinivas
authors.forename: Manoranjan
authors.initials: M
authors.lastname: Mahapatra
authors.forename: V P
authors.initials: VP
authors.lastname: Choudhry
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000150:complications / Q000235:genetics; D002648:Child; D005260:Female; D042882:Gallstones / Q000097:blood / Q000209:etiology / Q000235:genetics; D006446:Hemoglobin E / Q000032:analysis / Q000235:genetics; D006451:Hemoglobin, Sickle / Q000032:analysis / Q000235:genetics; D006456:Hemoglobinuria / Q000097:blood / Q000150:complications / Q000235:genetics; D006801:Humans; D008297:Male; D012720:Severity of Illness Index
chemical_list: D006451:Hemoglobin, Sickle; D006446:Hemoglobin E