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 che...

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Bibliographic Details
Published in:American journal of hematology 2007-07, Vol.82 (7), p.643-649
Main Authors: Masiello, David, Heeney, Matthew M., Adewoye, Adeboye H., Eung, Shawn H., Luo, Hong‐Yuan, Steinberg, Martin H., Chui, David. H.K.
Format: Article
Language:English
Subjects:
Adult
Anemia, Hemolytic, Congenital - genetics
Anemia, Hemolytic, Congenital - metabolism
Anemia, Hemolytic, Congenital - pathology
Anemia, Hemolytic, Congenital - therapy
Anemias. Hemoglobinopathies
Biological and medical sciences
Child
Diseases of red blood cells
Female
Hematologic and hematopoietic diseases
Hemoglobin E - genetics
Hemoglobin E - metabolism
Hemoglobin, Sickle - genetics
Hemoglobin, Sickle - metabolism
Humans
Infant
Infant, Newborn
Male
Medical sciences
Mutation - genetics
sickle cell disease
variant hemoglobin
β‐thalassemia
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Summary: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/β+‐thalassemia. Their hematological features and clinical course appear to parallel those of Hb S/β+‐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/β+‐thalassemia, and treated appropriately when they develop sickling‐related symptoms and complications. Am. J. Hematol., 2007. © 2007 Wiley‐Liss, Inc.
ISSN:0361-8609
1096-8652
DOI:10.1002/ajh.20847