A patient with hemoglobin S and elevated hemoglobin F may either have a serious hemoglobinopathy or a relatively benign condition.
Syndromes associated with hemoglobin S and elevated hemoglobin F:
(1) homozygous hemoglobin S (sickle cell disease)
(2) hemoglobin S and beta0-thalassemia
(3) heterozygous hemoglobin S (sickle cell trait) and hereditary persistence of hemoglobin F (HPHF)
In Blacks the most common form of hereditary persistence of hemoglobin F encountered is the heterozygous deletional trait, which is associated with pancellular (homocellular) distribution of the hemoglobin F.
Findings
Sickle Cell Disease or Sickle-Thalassemia
Sickle Cell Trait and Heterozygous HPHF
clinical
symptomatic, often from a young age
benign
percent hemoglobin F
5 to 30%
20 to 40%
distribution of hemoglobin F in RBCs
heterocellular
homocellular
The presence of sickle cell trait and the nondeletional form of hemoglobin F would give a hemoglobin F level comparable to sickle cell disease or sickle-thalassemia. It could also show a heterocellular distribution of hemoglobin F. However, it would have a relatively benign course.
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