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Related Experiment Videos

Temporal sequence of splenic dysfunction in sickle cell disease.

A D Adekile1, A Owunwanne, K Al-Za'abi

  • 1Department of Pediatrics, Faculty of Medicine, Kuwait University. adekile@hsc.kuniv.edu.kw

American Journal of Hematology
|February 9, 2002
PubMed
Summary

Sickle cell disease patients lose spleen function early. Phagocytic function declines first, while spleen filtration function persists longer, impacting disease management.

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Area of Science:

  • Pediatric Hematology
  • Nuclear Medicine
  • Medical Imaging

Background:

  • Sickle cell disease (SCD) commonly leads to early splenic dysfunction.
  • Technetium-99m colloid scintigraphy often fails to visualize the spleen in SCD patients.
  • Preliminary studies suggest technetium-99m heat-denatured RBC scintigraphy may visualize the spleen when colloid uptake is absent.

Purpose of the Study:

  • To investigate the sequential loss of splenic function in pediatric sickle cell disease patients.
  • To differentiate between phagocytic and filtration functions of the spleen using different scintigraphic agents.
  • To correlate splenic function with hematological parameters and alpha-thalassemia trait.

Main Methods:

  • Studied 46 pediatric patients (2-16 years) with sickle cell disease (36 SS, 7 Sbeta(0) thal, 3 SD).

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  • Assessed splenic function using technetium-99m colloid scintigraphy (evaluating phagocytic function).
  • Utilized technetium-99m heat-denatured RBC scintigraphy (evaluating filtration function) in select patients.
  • Compared spleen visualization and uptake patterns across different patient groups.

    • Main Results:

    • 18 patients (39.1%) had normal splenic colloid uptake, 15 (32.6%) partial, and 13 (28.3%) absent.
    • Patients with normal colloid uptake had significantly higher mean hemoglobin (10.2 g/dl) compared to those with partial/absent uptake (9.0 g/dl).
    • Heat-denatured RBC scintigraphy visualized the spleen in all Group 2 patients and 4/12 Group 3 patients, indicating persistent filtration function.

    Conclusions:

    • Splenic phagocytic function, assessed by colloid uptake, is lost earlier than splenic filtration function, assessed by denatured RBC uptake, in sickle cell disease.
    • The persistence of filtration function may have implications for understanding disease symptomatology and developing therapeutic strategies.
    • Co-existing alpha-thalassemia trait was significantly more prevalent in patients with normal or partial splenic uptake compared to absent uptake.