Hb SE disease: a clinico-hematological profile.
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.
Mishra
Pravas
P
Department of Hematology, All-India Institute of Medical Sciences, New Delhi, India.
Pati
H P
HP
Chatterjee
Tathagat
T
Dixit
Ashish
A
Choudhary
Dharma R
DR
Srinivas
M Upendra
MU
Mahapatra
Manoranjan
M
Choudhry
V P
VP
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
D006451:Hemoglobin, Sickle; D006446:Hemoglobin E