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Hemoglobin SE disease—A concise review
Author(s) -
Masiello David,
Heeney Matthew M.,
Adewoye Adeboye H.,
Eung Shawn H.,
Luo HongYuan,
Steinberg Martin H.,
Chui David. H.K.
Publication year - 2007
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.20847
Subject(s) - medicine , thalassemia , disease , pediatrics , acute chest syndrome , anemia , hemoglobinopathy , population , hemoglobin , sickle cell anemia , environmental health
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.