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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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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.

|April 15, 1995

Summary

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.

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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.
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.