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Related Experiment Videos

Functional asplenia in hemoglobin SC disease

P A Lane1, J L O'Connell, J L Lear

  • 1Colorado Sickle Cell Treatment and Research Center, University of Colorado School of Medicine, Denver, USA.

Blood
|April 15, 1995
PubMed
Summary
This summary is machine-generated.

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Functional asplenia is common in sickle-hemoglobin C (SC) disease, developing after age 4. Red blood cell pit counts reliably assess splenic function in SC disease, informing penicillin prophylaxis decisions.

Area of Science:

  • Hematology
  • Pediatric Medicine
  • Immunology

Background:

  • Sickle-hemoglobin C (SC) disease can lead to functional asplenia and increased risk of septicemia.
  • Prophylactic penicillin use in SC disease is debated due to lack of defined splenic function incidence.
  • Red blood cell pit count is a validated splenic function assay in other disorders.

Purpose of the Study:

  • To define the incidence of functional asplenia in sickle-hemoglobin C (SC) disease.
  • To validate the red blood cell pit count assay for splenic function in SC disease.
  • To inform the use of prophylactic penicillin in SC disease.

Main Methods:

  • Prospective, multicenter study of 201 subjects with SC disease (6 months to 90 years).
  • Assessment of splenic function using red blood cell pit counts and radionuclide liver-spleen scans in 43 subjects.

Related Experiment Videos

  • Comparison of pit counts in SC disease with splenectomized subjects without hemoglobinopathy or with sickle cell anemia.

    • Main Results:

    • Functional asplenia, indicated by pit counts >20%, was observed in 22% of subjects aged 4-12 and 45% of those >12 years.
    • Pit counts were significantly higher in splenectomized SC disease patients compared to other groups (P < .001).
    • Two non-splenectomized SC disease patients with high pit counts died from pneumococcal septicemia.

    Conclusions:

    • Functional asplenia occurs in SC disease, typically developing after age 4.
    • Red blood cell pit count is a reliable splenic function measure in SC disease, with higher thresholds for asplenia.
    • Routine penicillin prophylaxis may not be necessary for infants and young children with SC disease.