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Does Elevated Hemoglobin F Modulate the Phenotype in Hb SD-Los Angeles?

Does Elevated Hemoglobin F Modulate the Phenotype in Hb SD-Los Angeles? Hemoglobin (Hb) SD-Los Angeles compound heterozygotes usually have a severe clinical course although the effect of an elevated Hb F on the clinical phenotype has not been reported previously. We describe 5 Kuwaiti children with Hb SD associated with Hb F levels >20%. They all presented with sickling-related symptoms by ≤2 years of age and have been followed for 3–15 years. All had severe clinical courses marked by varying degrees of splenic sequestration crises, acute chest syndrome, vaso-occlusive crises, osteomyelitis and avascular necrosis of the femoral head. This pattern is in contrast with the usually mild presentation in Kuwaiti Hb SS patients with elevated Hb F. It therefore appears that Hb F does not ameliorate the clinical phenotype in Hb SD-Los Angeles. The reasons for this are not quite clear. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Acta Haematologica Karger

Does Elevated Hemoglobin F Modulate the Phenotype in Hb SD-Los Angeles?

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References (27)

Publisher
Karger
Copyright
© 2010 S. Karger AG, Basel
ISSN
0001-5792
eISSN
1421-9662
DOI
10.1159/000276998
Publisher site
See Article on Publisher Site

Abstract

Hemoglobin (Hb) SD-Los Angeles compound heterozygotes usually have a severe clinical course although the effect of an elevated Hb F on the clinical phenotype has not been reported previously. We describe 5 Kuwaiti children with Hb SD associated with Hb F levels >20%. They all presented with sickling-related symptoms by ≤2 years of age and have been followed for 3–15 years. All had severe clinical courses marked by varying degrees of splenic sequestration crises, acute chest syndrome, vaso-occlusive crises, osteomyelitis and avascular necrosis of the femoral head. This pattern is in contrast with the usually mild presentation in Kuwaiti Hb SS patients with elevated Hb F. It therefore appears that Hb F does not ameliorate the clinical phenotype in Hb SD-Los Angeles. The reasons for this are not quite clear.

Journal

Acta HaematologicaKarger

Published: Jan 1, 2010

Keywords: Avascular necrosis; Hb F; Hb SD-Los Angeles; Hemoglobin; Sickle cell disease; Vaso-occlusive crises

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