Hemoglobin SE disease: a concise review.
An infant with Hb SE disease is reported. He was clinically well. Review of the literature shows that patients aged 18 and younger are usually well. On the other hand, more than half of those aged 20 and older developed sickling-related complications, including potentially life-threatening acute chest syndrome. These patients have 60-65% Hb S, similar to the percent Hb S in patients with Hb S/beta(+)-thalassemia. Their hematological features and clinical course appear to parallel those of Hb S/beta(+)-thalassemia. Patients have variable levels of anemia, and some develop clinical complications. With population migrations and increasing racial intermarriages, Hb SE disease is expected to be encountered more often around the globe. Patients with Hb SE disease should be followed and managed in a similar fashion as those with Hb S/beta(+)-thalassemia, and treated appropriately when they develop sickling-related symptoms and complications.
Masiello
David
D
The Center of Excellence in Sickle Cell Disease, Department of Medicine, School of Medicine, Boston University, Boston, MA, USA.
Heeney
Matthew M
MM
Adewoye
Adeboye H
AH
Eung
Shawn H
SH
Luo
Hong-Yuan
HY
Steinberg
Martin H
MH
Chui
David H K
DH
D000328:Adult; D000745:Anemia, Hemolytic, Congenital / Q000235:genetics / Q000378:metabolism* / Q000473:pathology* / Q000628:therapy; D002648:Child; D005260:Female; D006446:Hemoglobin E / Q000235:genetics / Q000378:metabolism*; D006451:Hemoglobin, Sickle / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009154:Mutation / Q000235:genetics
D006451:Hemoglobin, Sickle; D006446:Hemoglobin E