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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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Hemoglobin SE disease: a concise review

David Masiello1, Matthew M Heeney, Adeboye H Adewoye

  • 1The Center of Excellence in Sickle Cell Disease, Department of Medicine, School of Medicine, Boston University, Boston, MA, USA.

American Journal of Hematology
|February 6, 2007
PubMed

Insights

Hemoglobin SE (Hb SE) disease is often mild in children but can cause serious complications in adults. Management should mirror that of Hb S/beta(+)-thalassemia.

Area of Science:

  • Hematology
  • Genetics
  • Pediatrics

Background:

  • Hemoglobin SE (Hb SE) disease is a hemoglobinopathy.
  • It arises from compound heterozygosity for Hb S and Hb E.
  • This condition is increasingly recognized globally due to population migration.

Observation:

  • A case of an infant with Hb SE disease who was clinically well is presented.
  • Literature review indicates that individuals under 18 with Hb SE disease are typically asymptomatic.
  • Conversely, over 50% of patients aged 20 and older experience sickling-related complications.

Findings:

  • Hb SE disease patients exhibit 60-65% Hb S, comparable to Hb S/beta(+)-thalassemia.
  • Hematological parameters and clinical progression in Hb SE disease resemble those in Hb S/beta(+)-thalassemia.
  • Variable anemia and the potential for clinical complications are noted in Hb SE disease.

Implications:

  • Hb SE disease is expected to be diagnosed more frequently worldwide.
  • Management strategies for Hb SE disease should align with those for Hb S/beta(+)-thalassemia.
  • Prompt treatment of sickling-related symptoms and complications is crucial for patients with Hb SE disease.

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