Functional asplenia in hemoglobin SC disease

P. A. Lane, J. L. O'Connell, J. L. Lear, Z. R. Rogers, G. M. Woods, K. L. Hassell, D. L. Wethers, D. W. Luckey, G. R. Buchanan

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Abstract

The incidence of functional asplenia in sickle-hemoglobin C (SC) disease has not been defined, and the use of prophylactic penicillin to prevent life- threatening septicemia in this disorder is controversial. The percentage of red blood cells with pits (pit count) is a reliable assay of splenic function in other disorders but has not been validated in hemoglobin SC disease. To address these issues, we conducted a prospective, multicenter study of splenic function in persons with hemoglobin SC disease. Baseline clinical data were recorded, and red blood cell pit counts were performed on 201 subjects, aged 6 months to 90 years, with hemoglobin SC; 43 subjects underwent radionuclide liver-spleen scanning. Pit counts greater than 20% were associated with functional asplenia as assessed by liver-spleen scan, whereas pit counts less than 20% were found in subjects with preserved splenic function. Pit counts greater than 20% were present in 0 of 59 subjects (0%) less than 4 years of age, in 19 of 86 subjects (22%) 4 to 12 years of age, and in 25 of 56 subjects (45%) greater than 12 years of age. Other subjects with hemoglobin SC, who had previously undergone surgical splenectomy, had higher pit counts (59.7% ± 9.5%) than splenectomized subjects without hemoglobinopathy (38.5% ± 8.8%) or with sickle cell anemia (20.5% ± 1.9%; P < .001). Two subjects with hemoglobin SC disease (not splenectomized), ages 14 and 15 years, with pit counts of 40.3% and 41.7% died from pneumococcal septicemia. These data indicate that functional asplenia occurs in many patients with hemoglobin SC disease, but its development is usually delayed until after 4 years of age. The pit count is a reliable measure of splenic function in hemoglobin SC disease, but values indicative of functional asplenia (>20% in our laboratory) are higher than in other disorders. The routine administration of prophylactic penicillin to infants and young children with hemoglobin SC disease may not be necessary.

Original languageEnglish (US)
Pages (from-to)2238-2244
Number of pages7
JournalBlood
Volume85
Issue number8
StatePublished - 1995

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Hemoglobin C Disease
Hemoglobin C
Sickle Hemoglobin
Hemoglobin
Hemoglobins
Penicillins
Liver
Blood
Spleen
Cells
Hemoglobinopathies
Erythrocyte Count
Sickle Cell Anemia
Splenectomy
Radioisotopes
Multicenter Studies
Assays
Sepsis
Erythrocytes
Prospective Studies

ASJC Scopus subject areas

  • Hematology

Cite this

Lane, P. A., O'Connell, J. L., Lear, J. L., Rogers, Z. R., Woods, G. M., Hassell, K. L., ... Buchanan, G. R. (1995). Functional asplenia in hemoglobin SC disease. Blood, 85(8), 2238-2244.

Functional asplenia in hemoglobin SC disease. / Lane, P. A.; O'Connell, J. L.; Lear, J. L.; Rogers, Z. R.; Woods, G. M.; Hassell, K. L.; Wethers, D. L.; Luckey, D. W.; Buchanan, G. R.

In: Blood, Vol. 85, No. 8, 1995, p. 2238-2244.

Research output: Contribution to journalArticle

Lane, PA, O'Connell, JL, Lear, JL, Rogers, ZR, Woods, GM, Hassell, KL, Wethers, DL, Luckey, DW & Buchanan, GR 1995, 'Functional asplenia in hemoglobin SC disease', Blood, vol. 85, no. 8, pp. 2238-2244.
Lane PA, O'Connell JL, Lear JL, Rogers ZR, Woods GM, Hassell KL et al. Functional asplenia in hemoglobin SC disease. Blood. 1995;85(8):2238-2244.
Lane, P. A. ; O'Connell, J. L. ; Lear, J. L. ; Rogers, Z. R. ; Woods, G. M. ; Hassell, K. L. ; Wethers, D. L. ; Luckey, D. W. ; Buchanan, G. R. / Functional asplenia in hemoglobin SC disease. In: Blood. 1995 ; Vol. 85, No. 8. pp. 2238-2244.
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abstract = "The incidence of functional asplenia in sickle-hemoglobin C (SC) disease has not been defined, and the use of prophylactic penicillin to prevent life- threatening septicemia in this disorder is controversial. The percentage of red blood cells with pits (pit count) is a reliable assay of splenic function in other disorders but has not been validated in hemoglobin SC disease. To address these issues, we conducted a prospective, multicenter study of splenic function in persons with hemoglobin SC disease. Baseline clinical data were recorded, and red blood cell pit counts were performed on 201 subjects, aged 6 months to 90 years, with hemoglobin SC; 43 subjects underwent radionuclide liver-spleen scanning. Pit counts greater than 20{\%} were associated with functional asplenia as assessed by liver-spleen scan, whereas pit counts less than 20{\%} were found in subjects with preserved splenic function. Pit counts greater than 20{\%} were present in 0 of 59 subjects (0{\%}) less than 4 years of age, in 19 of 86 subjects (22{\%}) 4 to 12 years of age, and in 25 of 56 subjects (45{\%}) greater than 12 years of age. Other subjects with hemoglobin SC, who had previously undergone surgical splenectomy, had higher pit counts (59.7{\%} ± 9.5{\%}) than splenectomized subjects without hemoglobinopathy (38.5{\%} ± 8.8{\%}) or with sickle cell anemia (20.5{\%} ± 1.9{\%}; P < .001). Two subjects with hemoglobin SC disease (not splenectomized), ages 14 and 15 years, with pit counts of 40.3{\%} and 41.7{\%} died from pneumococcal septicemia. These data indicate that functional asplenia occurs in many patients with hemoglobin SC disease, but its development is usually delayed until after 4 years of age. The pit count is a reliable measure of splenic function in hemoglobin SC disease, but values indicative of functional asplenia (>20{\%} in our laboratory) are higher than in other disorders. The routine administration of prophylactic penicillin to infants and young children with hemoglobin SC disease may not be necessary.",
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