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Related Experiment Videos

Spleen dysfunction in hemoglobinopathies determined by pitted red cells.

R Grover1, D L Wethers

  • 1Comprehensive Sickle Cell Program, St. Luke's/Roosevelt Hospital Center, New York, New York.

The American Journal of Pediatric Hematology/Oncology
|January 1, 1988
PubMed
Summary

Pitted red cell counts indicate splenic dysfunction in hemoglobinopathies. Counts increase with age in sickle cell disease and markedly in CC disease, but remain normal in S beta+ thalassemia.

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Area of Science:

  • Hematology
  • Pediatrics
  • Immunology

Background:

  • Hemoglobinopathies like sickle cell disease (SS), SC disease, S beta-thalassemia, and CC disease are associated with splenic dysfunction.
  • Pitted red cells (pit) are a reliable indicator of splenic dysfunction.

Purpose of the Study:

  • To investigate splenic dysfunction using pitted red cell counts in various hemoglobinopathies.
  • To correlate splenic function with age, steady state, and specific clinical events.

Main Methods:

  • Quantification of pitted red cells (pit count) in patients with SS, SC, S beta-thalassemia, and CC hemoglobinopathies.
  • Analysis of pit counts in relation to patient age, disease state (steady state), and clinical events (pneumococcal sepsis, osteomyelitis, splenic sequestration crisis).

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Main Results:

  • Pit counts generally increased with age in steady-state SS disease.
  • A significant rise in pit counts was observed in patients with CC disease.
  • Pit counts remained normal in S beta+ thalassemia patients across all ages.
  • Elevated pit counts were universal in children with SS disease during pneumococcal sepsis.
  • Pit counts were elevated in most SS disease patients with osteomyelitis.
  • Pit counts were normal at the onset and during treatment of acute splenic sequestration crisis in children.
  • No correlation between pit count and spleen size was found in infants under one year.

Conclusions:

  • Pitted red cell counts are valuable for assessing splenic function in hemoglobinopathies.
  • Splenic dysfunction varies significantly among different hemoglobinopathy types and clinical conditions.
  • Age and specific infections like pneumococcal sepsis are associated with increased splenic dysfunction in SS disease.