Hb SE disease: a clinico-hematological profile

Ann Hematol. 2005 Oct;84(10):667-70. doi: 10.1007/s00277-005-1044-2. Epub 2005 Jun 10.

Abstract

Hb S and Hb E are globally common hemoglobinopathies. However, Hb SE double heterozygous state is uncommon, with only 25 cases reported so far in literature. We present two more cases. One presented with gallstones, and the other was asymptomatic. This type of disorder was previously described as a relatively asymptomatic condition compared to HbSS. A review of the 25 reported cases in literature shows that 40.7% (11/27) of these cases are symptomatic. Gender, hematological parameters and levels of Hb S, E or F do not predict clinical severity.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Anemia, Sickle Cell* / blood
  • Anemia, Sickle Cell* / complications
  • Anemia, Sickle Cell* / genetics
  • Child
  • Female
  • Gallstones / blood
  • Gallstones / etiology
  • Gallstones / genetics
  • Hemoglobin E* / analysis
  • Hemoglobin E* / genetics
  • Hemoglobin, Sickle* / analysis
  • Hemoglobin, Sickle* / genetics
  • Hemoglobinuria* / blood
  • Hemoglobinuria* / complications
  • Hemoglobinuria* / genetics
  • Humans
  • Male
  • Severity of Illness Index

Substances

  • Hemoglobin, Sickle
  • Hemoglobin E